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Classification and Diagnostic Criteria


The unique role of criteria sets for classification and diagnosis in rheumatology reflects the clinical and scientific challenges characteristic of rheumatic diseases. Rheumatic diseases often cannot be defined by a single clinical, laboratory, radiologic, or pathologic feature because of their complicated and heterogeneous nature. Consequently, in the absence of a “gold standard” test, most rheumatic disorders are diagnosed or classified depending on a combination of findings. The process of generating criteria sets involves identifying such common findings that both are indicative of the given disease (sensitivity) and demarcate it from other diseases (specificity). As a means to facilitate standardization, the development of criteria sets has long been a major facet of rheumatologic research. Accordingly, a lot of progress has been made in this field, especially in recent decades.

Whereas early on many criteria were mostly based on expert opinion or used small numbers of patients, the approach to criteria formulation has evolved greatly over the years as experience with the derivation of criteria sets as well as progress in measurement sciences in general has fostered improvements in methodologic strategies.

This has recently led to the proposal of standards for the development and validation of criteria sets. To meet these standards, the list of possible items, which should be selected by appropriate consensus methodology, undergo quantitative validity testing, as well as evaluation of all items for reliability, precision, and availability. Apart from the quantitative requirements of a minimum of 100 cases and controls each, there are important considerations regarding the choice of cases and controls because cases should represent all degrees of disease severity. Second, because prior criteria sets often recruited all their cases from academic medical centers, sets also intended for use in epidemiologic studies should aim to include both clinical and community cases to be more representative of the general population. The proper choice of controls should also be informed by the intended purpose of the criteria set (e.g., whether it should distinguish patients with the disease from patients without the disease or from patients with other rheumatic diseases) and should ideally include multiple groups reflecting the desired discriminatory ability. Finally, all criteria sets should be validated in an independent cohort and by investigators who did not participate in the development to avoid bias and circularity of reasoning.

However, because many of the currently used criteria were published before these standards were published, there is significant variability in the methodology used for development and validation. Furthermore, decades of experience with some of the frequently used criteria have revealed a potential for improvement in many criteria sets. Additionally, progress in the general understanding of many rheumatic diseases as well as new diagnostic possibilities have prompted the revision of other criteria. Progress in therapeutic modalities has also had an impact on criteria sets because of better safety profiles in the context of recent studies, suggesting early intervention possibly being able to alter long-term prognosis in some diseases. This has induced efforts to improve sensitivity to capture patients with earlier disease.

Consequently, the field of rheumatologic criteria is currently undergoing fast-paced changes, with many criteria having been revised in recent years and many more currently being in the process of reevaluation. Although these are certainly welcome developments, it has become increasingly difficult to oversee the wide field of criteria sets in rheumatology as a result. This was the rationale to write this chapter, wherein we aimed to help get an overview of the current state of the ever more complicated landscape of criteria sets in rheumatology.

Considering that this appendix contains both classification and diagnostic criteria sets, it is of critical importance to note a few relevant distinctions among the different types of criteria.

Classification criteria are developed with the primary intent of ensuring comparability in clinical research by facilitating the formation of patient cohorts that have the most important manifestations of a given disease and are as homogeneous as possible, which requires criteria sets to be highly specific. Second, as classification criteria are commonly used in clinical research, another important rationale for the requirement of high specificity is to ensure that as few patients without the disease (“false positives”) as possible are exposed to experimental interventions. However, because there is always a “trade-off” between sensitivity and specificity, a strict focus on specificity comes at the cost of reduced sensitivity, that is, the increased risk of excluding patients who actually have the given disease (“false negatives”). Because patients with uncommon presentations or patients early in their disease history may be missed in this manner, classification criteria may not depict all the heterogeneity of complex diseases. Given these characteristics, even though they are often used this way, criteria sets designed for classification should only be used with caution for diagnostic purposes in a clinical setting. Moreover, a review of criteria set methodology by Johnson et al. showed that the performance of most classification criteria sets as diagnostic tools has not been evaluated and some criteria sets that were evaluated performed poorly in this setting.

Notably, the development of criteria sets for diagnosis is very challenging for numerous reasons. Whereas classification criteria strive to achieve homogeneity in research cohorts across the world, diagnostic criteria need to depict the whole broad spectrum of manifestations of a given disease while still offering high specificity. Considering these more ambitious requirements, the performance of diagnostic criteria can be highly variable depending on geographic region, ethnicity, and even the practice setting (e.g., general practice vs academic medical center) in which the criteria are used because of variations in pretest odds. Therefore especially for rare conditions, the development of diagnostic criteria that capture all facets of a given disease while retaining very high specificity and being universally applicable may not be attainable except in diseases with a true diagnostic “gold standard” or well-understood pathology and etiology. Accordingly, as noted earlier, to date, few diagnostic criteria sets for rheumatic diseases have been validated according to current methodologic standards. A diagnosis can thus only be made by considering not only the features represented in a criteria set but the whole individual presentation in the context of the above-mentioned factors determining pretest probability, as well as the probability of differential diagnoses and their risks and treatment implications.

With regard to the limitations discussed, the American College of Rheumatology has decided to no longer consider diagnostic criteria but only classification criteria for endorsement while still appreciating the need for new tools assisting diagnosis in clinical practice. Interestingly, Yazici et al. have proposed the development of “tailored” criteria sets for specific subspecialties and practice settings, which would elude some of the problems with pretest probability variations, decrease the differential diagnoses, and therefore have the potential for better performance.

In conclusion, classification criteria play a critical role in rheumatology because they facilitate comparability of research data across the globe and thereby promote progress in research as well as in clinical care. However, in dealing with classification criteria, it is important to be mindful of their intended purpose and the resulting limitations in applicability. Because there was and is tremendous progress being made in this field, it has become harder to keep track of the status quo, which motivated us to depict the current landscape of rheumatologic criteria. We primarily included classification and diagnostic criteria endorsed by the relevant specialist medical societies but also incorporated proposed criteria for diseases in which no criteria could be validated, striving for a representation as comprehensive as possible. Given the already substantial extent of this appendix, even though we firmly acknowledge the importance of sensitivity and specificity data, we did not directly include them in the appendix but solely provided references to the original articles including those data.

With most criteria mentioned in the specific chapters, it was also unfeasible to provide all criteria sets together in the printed version of the textbook, but readers are directed to the respective online supplement of this book, which comprises all relevant criteria.

Classification And Diagnostic Criteria

  • ACUTE RHEUMATIC FEVER

    • Revised Jones Criteria

  • ADULT-ONSET STILL DISEASE

    • Comparison Among the Main Classification Criteria

  • ANTIPHOSPHOLIPID SYNDROME

    • Revised Classification Criteria for Antiphospholipid Syndrome

    • Preliminary Criteria for the Classification of Catastrophic APS

  • COMPLEX REGIONAL PAIN SYNDROME

    • International Association for the Study of Pain 2007 Proposed Diagnostic Criteria for Complex Regional Pain Syndrome

  • DIFFUSE IDIOPATHIC SKELETAL HYPEROSTOSIS

    • Published Criteria for the Diagnosis of Diffuse Idiopathic Skeletal Hyperostosis

    • Criteria for Early-Phase Diffuse Idiopathic Skeletal Hyperostosis

  • FIBROMYALGIA

    • 1990 American College of Rheumatology Criteria for the Classification of Fibromyalgia

    • 2010 American College of Rheumatology Preliminary Criteria for the Diagnosis of Fibromyalgia

    • 2016 Revisions to the 2010/2011 Fibromyalgia Diagnostic Criteria

  • GOUT

    • American College of Rheumatology/European League Against Rheumatism Gout Classification Criteria

    • Diagnostic Rule for Acute Gouty Arthritis in Primary Care Without Joint Fluid Analysis

  • HYPERMOBILITY

    • Beighton Criteria

  • IGG4-RELATED DISEASE

    • 2019 American College of Rheumatology/European League Against Rheumatism Classification Criteria for IgG4-Related Disease (IGG4-RD)

  • INFLAMMATORY MUSCLE DISEASE

    • Idiopathic Inflammatory Myopathies

      • The European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) Classification Criteria for Adult and Juvenile Idiopathic Inflammatory Myopathies (IIMs)

      • Components of Classification Criteria for the Idiopathic Inflammatory Myopathies (except Inclusion Body Myositis )

      • Myositis Study Group and European Neuromuscular Centre Workshop–Approved Classification Criteria for the Idiopathic Inflammatory Myopathies

    • Inclusion Body Myositis

      • “Griggs Criteria” for Inclusion Body Myositis

      • The European Neuromuscular Centre Inclusion Body Myositis 2011 Research Diagnostic Criteria

    • Polymyositis and Dermatomyositis

      • Bohan and Peter Criteria for the Diagnosis of Polymyositis and Dermatomyositis

  • JUVENILE IDIOPATHIC ARTHRITIS

    • International League of Associations for Rheumatology Classification for Juvenile Idiopathic Arthritis

    • Pediatric Rheumatology International Trials Organization (PRINTO) 2019 Preliminary Criteria for Juvenile Idiopathic Arthritis (JIA)

    • Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis

      • Criteria for the Classification of Macrophage Activation Syndrome in Systemic Juvenile Idiopathic Arthritis

  • KASHIN-BECK DISEASE

    • Diagnostic Criteria for Kashin-Beck Disease

  • OSTEOARTHRITIS

    • 1986 American College of Rheumatology Criteria for the Classification of Knee Osteoarthritis

    • 1990 American College of Rheumatology Classification Criteria for Osteoarthritis of the Hand

    • 1991 American College of Rheumatology Criteria for the Classification of Osteoarthritis of the Hip

  • PSORIATIC ARTHRITIS

    • The CASPAR Criteria

  • REACTIVE ARTHRITIS

    • Diagnostic Criteria for Reactive Arthritis

  • RELAPSING POLYCHONDRITIS

    • Diagnostic Criteria for Relapsing Polychondritis

  • RHEUMATOID ARTHRITIS

    • 2010 American College of Rheumatology/European League Against Rheumatism Classification Criteria for Rheumatoid Arthritis

  • SJÖGREN SYNDROME

    • 2016 American College of Rheumatology/European League Against Rheumatism Classification Criteria for Primary Sjögren Syndrome

    • 2012 American College of Rheumatology Proposed Classification Criteria for Sjögren Syndrome

  • SPONDYLOARTHRITIS

    • Axial

      • Assessment of Spondyloarthritis International Society Criteria for Axial Spondylarthritis

      • Specification of the Variables Used for the Assessment of SpondyloArthritis International Society Criteria for Classification of Axial Spondyloarthritis

      • Comparison of Various Criteria for the Diagnosis of Inflammatory Back Pain

    • Peripheral

      • Assessment of SpondyloArthritis International Society Classification Criteria for Peripheral Spondyloarthritis

      • Combined Use of the Assessment of SpondyloArthritis International Society Criteria for Axial and Peripheral Spondyloarthritis in the Entire Spondyloarthritis Population

      • Definition of Spondyloarthritis Features for Use of Assessment of SpondyloArthritis International Society Classification Criteria for Peripheral Spondyloarthritis

  • SYSTEMIC LUPUS ERYTHEMATOSUS

    • Systemic Lupus International Collaborating Clinics Classification Criteria for Systemic Lupus Erythematosus

    • 1997 Update of the 1982 American College of Rheumatology Revised Criteria for the Classification of Systemic Lupus Erythematosus

    • 2019 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Systemic Lupus Erythematosus (SLE)

  • SYSTEMIC SCLEROSIS AND SCLERODERMA

    • 2013 American College of Rheumatology/European League Against Rheumatism Criteria for the Classification of Systemic Sclerosis

    • Definitions of Items and Subitems in the American College of Rheumatology/European League Against Rheumatism Criteria for the Classification of Systemic Sclerosis

  • VASCULITIS

    • Definitions for Vasculitides Adopted by the 2012 International Chapel Hill Consensus Conference on the Nomenclature of Vasculitides (CHCC2012)

    • Dermatologic Addendum to the 2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides

    • Antineutrophil Cytoplasmic Antibody–Associated Vasculitis

      • Entry Criteria and Definitions

      • Classification Algorithm for the Classification of the Antineutrophil Cytoplasmic Antibody–Associated Vasculitides and Polyarteritis Nodosa

  • Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss Syndrome)

      • 1990 American College of Rheumatology Criteria for the Classification of Churg-Strauss Syndrome

      • Proposed Revision of the Nomenclature of Eosinophilic Granulomatosis With Polyangiitis (EGPA) and a New Proposed Entity Referred to as Hypereosinophilic Asthma With Systemic (Nonvasculitic) Manifestations

      • Granulomatosis With Polyangiitis

      • 1990 American College of Rheumatology Criteria for the Classification of Granulomatosis With Polyangiitis (Wegener Granulomatosis)

      • ACR/EULAR Provisional 2017 Classification Criteria for Granulomatosis With Polyangiitis (GPA)

      • Childhood Wegener Granulomatosis

      • Final European League Against Rheumatism/Pediatric Rheumatology International Trials Organisation/Paediatric Rheumatology European Society Childhood Wegener Granulomatosis Criteria (With Glossary) and Classification Definition

    • Polyarteritis Nodosa

      • 1990 American College of Rheumatology Criteria for the Classification of Polyarteritis Nodosa

      • Childhood Polyarteritis Nodosa

      • Final European League Against Rheumatism/Pediatric Rheumatology International Trials Organisation/Paediatric Rheumatology European Society Childhood Polyarteritis Nodosa Criteria (With Glossary) and Classification Definition

    • Behçet Disease

      • International Criteria for Behçet Disease

      • International Study Group Criteria for the Diagnosis of Behçet Disease

      • Japanese Criteria for the Diagnosis of Behçet Disease

      • Consensus Classification of Paediatric Behçet Disease (BD)

    • Cryoglobulinemia

      • Classification Criteria for Cryoglobulinemic Vasculitis

    • Henoch-Schönlein Purpura and Immunoglobulin A Vasculitis

      • Final European League Against Rheumatism/Pediatric Rheumatology International Trials Organisation/Paediatric Rheumatology European Society Henoch-Schönlein Purpura Criteria (With Glossary) and Classification Definition

      • 1990 American College of Rheumatology Criteria for the Classification of Henoch-Schönlein Purpura (Traditional Format)

      • Childhood Henoch-Schönlein Purpura

      • Final European League Against Rheumatism/Pediatric Rheumatology International Trials Organisation/Paediatric Rheumatology European Society HSP Criteria (With Glossary) and Classification Definition

    • Hypersensitivity Vasculitis

      • 1990 American College of Rheumatology Criteria and Definitions Used for the Classification of Hypersensitivity Vasculitis

    • Kawasaki Disease

      • Diagnosis of Classic Kawasaki Disease

      • European League Against Rheumatism/Paediatric Rheumatology European Society Classification Criteria for Kawasaki Disease

    • Polymyalgia Rheumatica and Giant Cell Arteritis

      • Polymyalgia Rheumatica

      • 2012 European League Against Rheumatism/American College of Rheumatology .

      • Provisional Classification Criteria for Polymyalgia Rheumatica

      • Giant Cell Arteritis

      • 1990 American College of Rheumatology Criteria for the Classification of Giant Cell (Temporal) Arteritis

    • Takayasu Arteritis

      • 1990 American College of Rheumatology Criteria for the Classification of Takayasu Arteritis

      • Childhood Takayasu Arteritis

      • Final European League Against Rheumatism/Pediatric Rheumatology International Trials Organisation/Paediatric Rheumatology European Society Childhood Takayasu Arteritis Criteria (with Glossary) and Classification Definition

      • Primary Angiitis of the Central Nervous System

      • Diagnostic Criteria for Primary Angiitis of the Central Nervous System (PACNS)

    • Periodic Fever Syndromes

      • Eurofever/PRINTO Classification Criteria for Hereditary Recurrent Fevers

      • Eurofever/PRINTO Clinical Classification Criteria for PFAPA and Hereditary Recurrent Fevers

    • Familial Mediterranean Fever

      • Criteria Set for Diagnosis of Familial Mediterranean Fever

      • Criteria for the Diagnosis of Familial Mediterranean Fever in Childhood

Acute Rheumatic Fever

Revised criteria can be found in Gewitz MH, Baltimore RS, Tani LY, et al; American Heart Association Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease of the Council on Cardiovascular Disease in the Young. Revision of the Jones Criteria for the diagnosis of acute rheumatic fever in the era of Doppler echocardiography: a scientific statement from the American Heart Association. Circulation . 2015;19;131(20):1806–1818.

The previously updated criteria were published in Dajani AS, Ayoub E, Bierman FZ, et al; Special Writing Group of the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease of the Council on Cardiovascular Disease in the Young of the American Heart Association. Guidelines for the diagnosis of rheumatic fever: Jones criteria, 1992 update [published correction appears in JAMA . 1993;269:476]. JAMA . 1992;268:2069–2073.

Adult-Onset Still Disease

Comparison Among the Main Classification Criteria
From Mahroum N, Mahagna H, Amital H. Diagnosis and classification of adult Still’s disease. J Autoimmun . 2014;48-49:34-37.
Cush et al. Yamaguchi et al. Fautrel et al.

  • 2 points each:

  • ■

    Quotidian fever >39°C

  • ■

    Evanescent rash

  • ■

    WBC count >12,000/mL and ESR >40 mm/1 hr

  • ■

    Negative ANA and RF

  • ■

    Carpal ankylosis

  • Major criteria:

  • ■

    Fever >39°C, intermittent, ≥1 week

  • ■

    Arthralgia ≥2 weeks

  • ■

    Characteristic rash

  • ■

    WBC count >10,000/mL (>80% granulocytes)

  • Major criteria:

  • ■

    Spiking fever ≥39°C

  • ■

    Arthralgia

  • ■

    Transient erythema

  • ■

    Pharyngitis

  • ■

    PMN leukocytes ≥80%

  • ■

    Glycosylated ferritin ≤20%

  • 1 point each:

  • ■

    Onset age >35 yr

  • ■

    Arthritis

  • ■

    Sore throat

  • ■

    RES involvement or LFT abnormal

  • ■

    Serositis

  • ■

    Cervical or tarsal ankylosis

  • Minor criteria:

  • ■

    Sore throat

  • ■

    Lymphadenopathy or splenomegaly

  • ■

    LFT abnormal

  • ■

    Negative ANA/RF

  • Minor criteria:

  • ■

    Maculopapular rash

  • ■

    WBC count >10, 000/mL

  • Probable AOSD: 10 pts during 12-wk observation

  • Definite AOSD: 10 pts with 6-mo observation

  • Exclusion criteria:

  • ■

    Infections

  • ■

    Malignancies

  • ■

    Rheumatic diseases

  • ■

    5 criteria: at least 2 major and 3 minor

  • 4 major criteria or 3 major + 2 minor

ANA , Antinuclear antibody; AOSD , adult-onset Still disease; ESR , erythrocyte sedimentation rate; LFT , liver function test; PMN , polymorphonuclear; RES , reticuloendothelial system; RF , rheumatic factor; WBC , white blood cell.

Antiphospholipid Syndrome

Revised Classification Criteria For Antiphospholipid Syndrome a
From Miyakis S, Lockshin MD, Atsumi T, et al. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost. 2006;4:295–306.

Antiphospholipid antibody syndrome (APS) is present if at least one of the clinical criteria and one of the laboratory criteria that follow are met. a

a Classification of APS should be avoided if less than 12 weeks or more than 5 years separates the positive aPL test and the clinical manifestation.

Clinical criteria

  • 1.

    Vascular thrombosis b

    b Coexisting inherited or acquired factors for thrombosis are not reasons for excluding patients from APS trials. However, two subgroups of APS patients should be recognized, according to (a) the presence and (b) the absence of additional risk factors for thrombosis. Indicative (but not exhaustive) such cases include age (>55 years in men and >65 years in women) and the presence of any of the established risk factors for cardiovascular disease (hypertension, diabetes mellitus, elevated LDL or low HDL cholesterol, cigarette smoking, family history of premature cardiovascular disease, BMI ≥30 kg m −2 , microalbuminuria, estimated GFR <60 mL min −1 , inherited thrombophilias, oral contraceptives, nephrotic syndrome, malignancy, immobilization, and surgery). Thus patients who fulfil criteria should be stratified according to contributing causes of thrombosis.

  • One or more clinical episodes c

    c A thrombotic episode in the past could be considered as a clinical criterion, provided that thrombosis is proved by appropriate diagnostic means and that no alternative diagnosis or cause of thrombosis is found.

    of arterial, venous, or small vessel thrombosis d

    d Superficial venous thrombosis is not included in the clinical criteria.

    in any tissue or organ. Thrombosis must be confirmed by objective validated criteria (i.e., unequivocal findings of appropriate imaging studies or histopathology). For histopathologic confirmation, thrombosis should be present without significant evidence of inflammation in the vessel wall.

  • 2.

    Pregnancy morbidity

    • (a)

      One or more unexplained deaths of a morphologically normal fetus at or beyond the 10th week of gestation with normal fetal morphology documented by ultrasound or by direct examination of the fetus or

    • (b)

      One or more premature births of a morphologically normal neonate before the 34th week of gestation because of (i) eclampsia or severe preeclampsia defined according to standard definitions or (ii) recognized features of placental insufficiency e

      e Generally accepted features of placental insufficiency include (i) abnormal or nonreassuring fetal surveillance test(s) (e.g., a nonreactive nonstress test, suggestive of fetal hypoxemia), (ii) abnormal Doppler flow velocimetry waveform analysis suggestive of fetal hypoxemia (e.g., absent end-diastolic flow in the umbilical artery), (iii) oligohydramnios (e.g., an amniotic fluid index of ≤5 cm), or (iv) a postnatal birth weight less than the 10th percentile for gestational age.

      or

    • (c)

      Three or more unexplained consecutive spontaneous abortions before the 10th week of gestation, with maternal anatomic or hormonal abnormalities and paternal and maternal chromosomal causes excluded.

In studies of populations of patients who have more than one type of pregnancy morbidity, investigators are strongly encouraged to stratify groups of subjects according to a, b, or c above f

f Investigators are strongly advised to classify patients with APS in studies into one of the following categories: I, more than one laboratory criterion present (any combination); IIa, LA present alone; IIb, aCL antibody present alone; and IIc, anti-β 2 glycoprotein-I antibody present alone.

.

Laboratory criteria f

  • 1.

    LA present in plasma on two or more occasions at least 12 weeks apart, detected according to the guidelines of the International Society on Thrombosis and Haemostasis (Scientific Subcommittee on LAs/phospholipid-dependent antibodies).

  • 2.

    aCL antibody of IgG and/or IgM isotype in serum or plasma, present in medium or high titer (i.e., >40 GPL or MPL, or > the 99th percentile), on two or more occasions, at least 12 weeks apart, measured by a standardized ELISA.

  • 3.

    Anti–β2 glycoprotein-I antibody of IgG and/or IgM isotype in serum or plasma (in titer > the 99th percentile), present on two or more occasions, at least 12 weeks apart, measured by a standardized ELISA, according to recommended procedures.

aCL , Anticardiolipin; BMI , body mass index; ELISA , enzyme-linked immunosorbent assay; GFR , glomerular filtration rate; GPL , IgG phospholipid unit; HDL , high-density lipoprotein; LA , lupus anticoagulant; LDL , low-density lipoprotein; MPL , IgM phospholipid unit.

Preliminary Criteria for the Classification of Catastrophic APS
From Asherson RA, Cervera R, de Groot PG, et al. Catastrophic antiphospholipid syndrome: international consensus statement on classification criteria and treatment guidelines. Lupus. 2003;12(7):530–534.

  • 1.

    Evidence of involvement of three or more organs, systems, and/or tissues a

    a Usually, clinical evidence of vessel occlusions, confirmed by imaging techniques when appropriate. Renal involvement is defined by a 50% rise in serum creatinine, severe systemic hypertension (>180/100 mm Hg), and/or proteinuria (>500 mg/24 hours).

  • 2.

    Development of manifestations simultaneously or in less than a week

  • 3.

    Confirmation by histopathology of small vessel occlusion in at least one organ or tissue b

    b For histopathologic confirmation, significant evidence of thrombosis must be present, although vasculitis may coexist occasionally.

  • 4.

    Laboratory confirmation of the presence of antiphospholipid antibodies (lupus anticoagulant and/or anticardiolipin antibodies) c

    c If the patient had not been previously diagnosed as having an APS, the laboratory confirmation requires that presence of antiphospholipid antibodies must be detected on two or more occasions at least 6 weeks apart (not necessarily at the time of the event), according to the proposed preliminary criteria for the classification of definite APS.

Definite catastrophic APS

  • ■

    All four criteria

Probable catastrophic APS

  • ■

    All four criteria, except for only two organs, systems, and/or tissues involvement

  • ■

    All four criteria, except for the absence of laboratory confirmation at least 6 weeks apart due to the early death of a patient never tested for aPL before the catastrophic APS

  • ■

    1, 2, and 4

  • ■

    1, 3, and 4 and the development of a third event in more than a week but less than a month, despite anticoagulation d

    d Wilson WA, Gharavi AE, Koike T et al. International consensus statement on preliminary classification criteria for definite antiphospholipid syndrome. Report of an international workshop. Arthritis Rheum. 1999;42: 1309–1311. APS , Antiphospholipid syndrome.

Complex Regional Pain Syndrome

International Association for the Study of Pain 2007 Proposed Diagnostic Criteria for Complex Regional Pain Syndrome
From Harden RN, Bruehl S, Stanton-Hicks M, Wilson PR. Proposed new diagnostic criteria for complex regional pain syndrome. Pain Med. 2007;8(4):326–331.

General definition of the syndrome

CRPS describes an array of painful conditions that are characterized by a continuing (spontaneous, evoked, or both) regional pain that is seemingly disproportionate in time or degree to the usual course of any known trauma or other lesion. The pain is regional (not in a specific nerve territory or dermatome) and usually has a distal predominance of abnormal sensory, motor, sudomotor, vasomotor, or trophic findings. The syndrome shows variable progression over time.

To make the clinical diagnosis, the following criteria must be met:

  • 1.

    Continuing pain that is disproportionate to any inciting event

  • 2.

    Must report at least one symptom in three of the four following categories:

    • ■

      Sensory: Reports of hyperesthesia or allodynia

    • ■

      Vasomotor: Reports of temperature asymmetry, skin color changes, or skin color asymmetry

    • ■

      Sudomotor or edema: Reports of edema, sweating changes, or sweating asymmetry

    • ■

      Motor or trophic: Reports of decreased range of motion, motor dysfunction (weakness, tremor, dystonia), or trophic changes (hair, nail, skin)

  • 3.

    Must display at least one sign at time of evaluation in two or more of the following categories:

    • ■

      Sensory: Evidence of hyperalgesia (to pinprick), allodynia (to light touch, temperature sensation, deep somatic pressure, or joint movement)

    • ■

      Vasomotor: Evidence of temperature asymmetry (>1°C), skin color changes, or asymmetry

    • ■

      Sudomotor or edema: Evidence of edema, sweating changes, or sweating asymmetry

    • ■

      Motor or trophic: Evidence of decreased range of motion, motor dysfunction (weakness, tremor, dystonia), or trophic changes (hair, nail, skin)

  • 4.

    There is no other diagnosis that better explains the signs and symptoms

For research purposes, the diagnostic decision rule should be at least one symptom in all four symptom categories and at least one sign (observed at evaluation) in two or more sign categories.

Diffuse Idiopathic Skeletal Hyperostosis

Published Criteria for the Diagnosis of Diffuse Idiopathic Skeletal Hyperostosis
From Resnick D, Niwayama G. Radiographic and pathologic features of spinal involvement in diffuse idiopathic skeletal hyperostosis (DISH). Radiology. 1976;119:559; and Utsinger PD. Diffuse idiopathic skeletal hyperostosis. Clin Rheum Dis. 1985;11:325.

Criteria of Resnick and Niwayama

  • 1.

    The presence of flowing calcification and ossification along the anterolateral aspect of at least four contiguous vertebral bodies with or without associated localized pointed excrescences at the intervening vertebral body–intervertebral disk junctions

  • 2.

    The presence of relative preservation of intervertebral disk height in the involved vertebral segment and the absence of extensive radiographic changes of “degenerative” disk disease, including vacuum phenomena and vertebral body marginal sclerosis

  • 3.

    The absence of apophyseal joint bony ankylosis and sacroiliac joint erosion, sclerosis, or intraarticular osseous fusion

Criteria of Utsinger

  • 1.

    Continuous ossification along the anterolateral aspect of at least four contiguous vertebral bodies, primarily in the thoracolumbar spine. Ossification begins as a fine, ribbon-like wave of bone but commonly develops into a broad, bumpy, buttress-like band of bone

  • 2.

    Continuous ossification along the anterolateral aspect of at least two contiguous vertebral bodies

  • 3.

    Symmetric and peripheral enthesopathy involving the posterior heel, superior patella, or olecranon, with the entheseal new bone having a well-defined cortical margin

Exclusions

  • I).

    Abnormal disk space height in the involved areas

  • II).

    Apophyseal joint ankylosis

Categories of diffuse idiopathic skeletal hyperostosis according to the Utsinger criteria are:

  • Definite = criterion 1

  • Probable = criteria 2 and 3

Criteria for Early-Phase Diffuse Idiopathic Skeletal Hyperostosis

Please see Kuperus JS, Oudkerk SF, Foppen W, et al. Criteria for early-phase diffuse idiopathic skeletal hyperostosis: development and validation. Radiology . 2019;291(2):420–426.

Fibromyalgia

1990 American College of Rheumatology Criteria for the Classification of Fibromyalgia
Modified from Wolfe F, Smythe HA, Yunus MB, et al. The American College of Rheumatology 1990 criteria for the classification of fibromyalgia: report of the multicenter criteria committee. Arthritis Rheum. 1990;33:160–172.

  • 1.

    History of widespread pain

    • Definition: Pain is considered widespread when all of the following are present: pain in the left side of the body, pain in the right side of the body, pain above the waist, and pain below the waist. In addition, axial skeletal pain (cervical spine or anterior chest or thoracic spine or low back) must be present. In this definition, shoulder and buttock pain is considered as pain for each involved side. “Low back” pain is considered lower segment pain.

  • 2.

    Pain in 11 of 18 tender point sites on digital palpation

    • Definition: Pain, on digital palpation, must be present in at least 11 of the following 18 sites:

    • Occiput: bilateral, at the suboccipital muscle insertions

    • Low cervical: bilateral, at the anterior aspects of the intertransverse spaces at C5–C7

    • Trapezius: bilateral, at the midpoint of the upper border

    • Supraspinatus: bilateral, at origins, above the scapula spine near the medial border

    • Second rib: bilateral, at the second costochondral junctions, just lateral to the junctions on upper surfaces

    • Lateral epicondyle: bilateral, 2 cm distal to the epicondyles

    • Gluteal: bilateral, in upper outer quadrants of buttocks in anterior fold of muscle

    • Greater trochanter: bilateral, posterior to the trochanteric prominence

    • Knee: bilateral, at the medial fat pad proximal to the joint line

    • Digital palpation should be performed with an approximate force of 4 kg.

    • For a tender point to be considered “positive,” the subject must state that the palpation was painful. “Tender” is not to be considered “painful.”

    • For classification purposes, patients will be said to have fibromyalgia if both criteria are satisfied. Widespread pain must have been present for at least 3 months.

    • The presence of a second clinical disorder does not exclude the diagnosis of fibromyalgia.

2010 American College of Rheumatology Preliminary Criteria for the Diagnosis of Fibromyalgia
From Wolfe F, Clauw DJ, Fitzcharles MA, et al. The American College of Rheumatology preliminary diagnostic criteria for fibromyalgia and measurement of symptom severity. Arthritis Care Res (Hoboken). 2010;62(5):600–610.

Criteria

A patient satisfies diagnostic criteria for fibromyalgia if the following three conditions are met:

  • 1.

    Widespread pain index (WPI) ≥7 and symptom severity (SS) scale score ≥5 or WPI 3–6 and SS scale score ≥9

  • 2.

    Symptoms have been present at a similar level for at least 3 months

  • 3.

    The patient does not have a disorder that would otherwise explain the pain

Ascertainment

  • 1.

    WPI: Note the number of areas in which the patient has had pain over the past week. In how many areas has the patient had pain? Score will be between 0 and 19.

    • Shoulder girdle, left

    • Shoulder girdle, right

    • Upper arm, left

    • Upper arm, right

    • Lower arm, left

    • Lower arm, right

    • Hip (buttock, trochanter), left

    • Hip (buttock, trochanter), right

    • Upper leg, left

    • Upper leg, right

    • Lower leg, left

    • Lower leg, right

    • Jaw, left

    • Jaw, right

    • Chest

    • Abdomen

    • Upper back

    • Lower back

    • Neck

  • 2.

    SS scale score:

    • Fatigue

    • Waking unrefreshed

    • Cognitive symptoms

    • For the each of the three symptoms above, indicate the level of severity over the past week using the following scale:

    • 0 = no problem

    • 1 = slight or mild problems, generally mild or intermittent

    • 2 = moderate, considerable problems, often present or at a moderate level

    • 3 = severe: pervasive, continuous, life-disturbing problems

    • Considering somatic symptoms in general, indicate whether the patient has a

      a Somatic symptoms that might be considered: muscle pain, irritable bowel syndrome, fatigue or tiredness, thinking or remembering problem, muscle weakness, headache, pain or cramps in the abdomen, numbness or tingling, dizziness, insomnia, depression, constipation, pain in the upper abdomen, nausea, nervousness, chest pain, blurred vision, fever, diarrhea, dry mouth, itching, wheezing, Raynaud phenomenon, hives or welts, ringing in ears, vomiting, heartburn, oral ulcers, loss of or change in taste, seizures, dry eyes, shortness of breath, loss of appetite, rash, sun sensitivity, hearing difficulties, easy bruising, hair loss, frequent urination, painful urination, and bladder spasms.

      :

    • 0 = no symptoms

    • 1 = few symptoms

    • 2 = a moderate number of symptoms

    • 3 = a great deal of symptoms

The SS scale score is the sum of the severity of the three symptoms (fatigue, waking unrefreshed, cognitive symptoms) plus the extent (severity) of somatic symptoms in general. The final score is between 0 and 12.

2016 Revisions to the 2010/2011 Fibromyalgia Diagnostic Criteria
From Wolfe F, Clauw DJ, Fitzcharles M-A, et al. 2016 Revisions to the 2010/2011 fibromyalgia diagnostic criteria. Semin Arthritis Rheum. 2016;46(3):319–329.

Criteria

A patient satisfies modified 2016 fibromyalgia criteria if the following three conditions are met:

  • 1.

    Widespread pain index (WPI) ≥7 and symptom severity scale (SSS) score ≥5 OR WPI of 4–6 and SSS score ≥9.

  • 2.

    Generalized pain, defined as pain in at least four of five regions, must be present. Jaw, chest, and abdominal pain are not included in generalized pain definition.

  • 3.

    Symptoms have been generally present for at least 3 months.

  • 4.

    A diagnosis of fibromyalgia is valid irrespective of other diagnoses. A diagnosis of fibromyalgia does not exclude the presence of other clinically important illnesses.

Ascertainment

  • 1.

    WPI: note the number of areas in which the patient has had pain over the last week. In how many areas has the patient had pain? Score will be between 0 and 19.

    • Left upper region (Region 1)

      • Jaw, left a

        a Not included in generalized pain definition.

      • Shoulder girdle, left

      • Upper arm, left

      • Lower arm, left

    • Right upper region (Region 2)

      • Jaw, right a

      • Shoulder girdle, right

      • Upper arm, right

      • Lower arm, right

    • Left lower region (Region 3)

      • Hip (buttock, trochanter), left

      • Upper leg, left

      • Lower leg, left

    • Right lower region (Region 4)

      • Hip (buttock, trochanter), right

      • Upper leg, right

      • Lower leg, right

    • Axial region (Region 5)

      • Neck

      • Upper back

      • Lower back

      • Chest a

      • Abdomen a

  • 2.

    Symptom severity (SS) scale score

    • Fatigue

    • Waking unrefreshed

    • Cognitive symptoms

      For the each of the three symptoms above, indicate the level of severity over the past week using the following scale:

    • 0 = no problem

    • 1 = slight or mild problems, generally mild or intermittent

    • 2 = moderate, considerable problems, often present and/or at a moderate level

    • 3 = severe: pervasive, continuous, life-disturbing problems

The SS scale score is the sum of the severity scores of the three symptoms (fatigue, waking unrefreshed, and cognitive symptoms) (0–9) plus the sum (0–3) of the number of the following symptoms the patient has been bothered by that occurred during the previous 6 months:

  • 1.

    Headaches (0–1)

  • 2.

    Pain or cramps in lower abdomen (0–1)

  • 3.

    And depression (0–1)

    • The final symptom severity score is between 0 and 12.

    • The fibromyalgia severity (FS) scale is the sum of the WPI and SS scale score.

    • The FS scale is also known as the polysymptomatic distress (PSD) scale.

Gout

American College of Rheumatology/European League Against Rheumatism Gout Classification Criteria a
From Neogi T, Jansen TL, Dalbeth N, et al. 2015 Gout classification criteria: an American College of Rheumatology/European League Against Rheumatism collaborative initiative. Arthritis Rheum . 2015;67(10):2557-2568.
Categories Score
Step 1: Entry criterion (only apply criteria below to those meeting this entry criterion) At least one episode of swelling, pain, or tenderness in a peripheral joint or bursa
Step 2: Sufficient criterion (if met, can classify as gout without applying criteria below) Presence of MSU crystals in a symptomatic joint or bursa (i.e., in synovial fluid) or tophus
Step 3: Criteria (to be used if sufficient criterion not met)
Clinical
Pattern of joint or bursa involvement during symptomatic episode(s) ever b Ankle or midfoot (as part of monoarticular or oligoarticular episode without involvement of the first metatarsophalangeal joint) 1
Involvement of the first metatarsophalangeal joint (as part of monoarticular or oligoarticular episode) 2
Characteristics of symptomatic episode(s) ever
Erythema overlying affected joint (patient reported or physician observed) One characteristic 1
Can’t bear touch or pressure to affected joint Two characteristics 2
Great difficulty with walking or inability to use affected joint Three characteristics 3

  • Time course of episode(s) ever

  • Presence (ever) of ≥2, irrespective of antiinflammatory treatment

Time to maximal pain <24 hours One typical episode 1
Resolution of symptoms in ≤14 days Recurrent typical episodes 2
Complete resolution (to baseline level) between symptomatic episodes
Clinical evidence of tophus
Draining or chalklike subcutaneous nodule under transparent skin, often with overlying vascularity, located in typical locations: joints, ears, olecranon bursae, finger pads, tendons (e.g., Achilles tendon) Present 4
Laboratory
Serum urate: Measured by uricase method. Ideally should be scored at a time when the patient was not receiving urate-lowering treatment and it was >4 weeks from the start of an episode (i.e., during intercritical period); if practicable, retest under those conditions. The highest value irrespective of timing should be scored. <4 mg/dL (<0.24 mmol/L) c −4
6–8 mg/dL (0.36–<0.48 mmol/L) 2
8–<10 mg/dL (0.48–<0.60 mmol/L) 3
≥10 mg/dL (≥0.60 mmol/L) 4
Synovial fluid analysis of a symptomatic (ever) joint or bursa (should be assessed by a trained observer) d MSU negative −2
Imaging e
Imaging evidence of urate deposition in symptomatic (ever) joint or bursa: ultrasound evidence of double-contour sign f or DECT demonstrating urate deposition. g Present (either modality) 4
Imaging evidence of gout-related joint damage: conventional radiography of the hands and/or feet demonstrates at least one erosion. h Present 4

a A web-based calculator can be accessed at http://goutclassificationcalculator.auckland.ac.nz and through the American College of Rheumatology (ACR) and European League Against Rheumatism (EULAR) websites.

b Symptomatic episodes are periods of symptoms that include any swelling, pain, and/or tenderness in a peripheral joint or bursa.

c If serum urate level is <4 mg/dL (<0.24 mmol/L), subtract 4 points; if serum urate level is ≥4–<6 mg/dL (≥0.24–<0.36 mmol/L), score this item as 0.

d If polarizing microscopy of synovial fluid from a symptomatic (ever) joint or bursa by a trained examiner fails to show monosodium urate monohydrate (MSU) crystals, subtract 2 points. If synovial fluid was not assessed, score this item as 0.

e If imaging is not available, score these items as 0.

f Hyperechoic irregular enhancement over the surface of the hyaline cartilage that is independent of the insonation angle of the ultrasound beam (note: false-positive double-contour sign [artifact] may appear at the cartilage surface but should disappear with a change in the insonation angle of the probe).

g The presence of color-coded urate at articular or periarticular sites. Images should be acquired using a dual-energy computed tomography (DECT) scanner, with data acquired at 80 and 140 kV and analyzed using gout-specific software with a two-material decomposition algorithm that color codes urate. A positive scan is defined as the presence of color-coded urate at articular or periarticular sites. Nailbed, submillimeter, skin, motion, beam hardening, and vascular artifacts should not be interpreted as DECT evidence of urate deposition.

h Erosion is defined as a cortical break with sclerotic margin and overhanging edge, excluding distal interphalangeal joints and gull’s wing appearance.

Diagnostic Rule for Acute Gouty Arthritis in Primary Care Without Joint Fluid Analysis
CPPD , Calcium pyrophosphate dihydrate deposition disease; MTP1 , first metatarsophalangeal joint.Modified from Kienhorst LBE, Janssens HJEM, Fransen J, Janssen M. The validation of a diagnostic rule for gout without joint fluid analysis: a prospective study. Rheumatology (Oxford). 2015;54(4):609–614.
Patient with monoarthritis
Male sex 2 points
Previous patient-reported arthritis attack 2 points
Onset within 1 day 0.5 points
Joint redness 1 point
MTP1 involvement 2.5 points
Hypertension or one or more cardiovascular diseases a 1.5 points
Serum uric acid level ≥5.88 mg/dL b 3.5 points
Total score

  • ≤4 points

  • ■

    no gout in 95%

  • ■

    consider different diagnoses like CPPD arthritis, reactive arthritis, septic arthritis, rheumatoid arthritis, osteoarthritis, or psoriatic arthritis

  • 5–7 points

  • ■

    uncertain diagnosis:

  • ■

    perform joint fluid aspiration and analysis with polarisation microscopy for the presence of crystals; extensive follow-up if this is not possible or available.

  • ≥8 points

  • ■

    gout in 87%

  • ■

    manage the patient as having gout including care for cardiovascular risk

a Indicates angina pectoris, myocardial infarction, heart failure, cerebrovascular accident, transient ischaemic attack, or peripheral vascular disease.

b Indicates a serum uric acid level of 5.88 mg/dL (equal to 0.35 mmol/L).

Hypermobility

Brighton Criteria

Major criteria

  • ■

    Beighton score of ≥4 1

    1. Beighton P, Horan F. Orthopaedic aspects of the Ehlers-Danlos syndrome. J Bone Joint Surg Br . 196951(3):444-4453.

  • ■

    Arthralgia for longer than 3 months in four or more joints

Minor criteria

  • ■

    Beighton score of 1, 2, or 3 1

  • ■

    Arthralgia (>3-month duration) in one to three joints or back pain (>3-month duration) or spondylosis, spondylolysis, or spondylolisthesis

  • ■

    Dislocation or subluxation in more than one joint or in one joint on more than one occasion

  • ■

    Three or more soft tissue lesions (e.g., epicondylitis, tenosynovitis, bursitis)

  • ■

    Marfanoid habitus: tall, slim, span greater than height (>1.03 ratio), upper segment less than lower segment (<0.89 ratio), arachnodactyly

  • ■

    Skin striae, hyperextensibility, thin skin, or abnormal scarring

  • ■

    Ocular signs: drooping eyelids, myopia, antimongoloid slant

  • ■

    Varicose veins, hernia, or uterine or rectal prolapse

  • ■

    Mitral valve prolapse

Requirement for diagnosis

Any one of the following:

  • ■

    Two major criteria

  • ■

    One major plus two minor criteria

  • ■

    Four minor criteria

  • ■

    Two minor criteria and unequivocally affected first-degree relative in family history

Modified from Grahame R. The revised (Brighton 1998) criteria for the diagnosis of benign joint hypermobility syndrome (BJHS). J Rheumatol. 2000;27:1777–1779.

Beighton Hypermobility Score
Adapted from Beighton P, Horan F. Orthopaedic aspects of the Ehlers-Danlos syndrome. J Bone Joint Surg Br. 1969;51(3):444–453 and https://www.shoulderdoc.co.uk/article/645 .
Joint Finding Points
Left little (fifth) finger Passive dorsiflexion beyond 90° 1
Passive dorsiflexion ≤90° 0
Right little (fifth) finger Passive dorsiflexion beyond 90° 1
Passive dorsiflexion ≤90° 0
Left thumb Passive dorsiflexion to the flexor aspect of the forearm 1
Cannot passively dorsiflex thumb to flexor aspect of the forearm 0
Right thumb Passive dorsiflexion to the flexor aspect of the forearm 1
Cannot passively dorsiflex thumb to flexor aspect of the forearm 0
Left elbow Hyperextends beyonds 10° 1
Extends ≤10 0
Right elbow Hyperextends beyond 10° 1
Extends ≤10 0
Left knee Hyperextends beyond 10° 1
Extends ≤10 0
Right knee Hyperextends beyond 10° 1
Extends ≤10 0
Forward flexion of trunk with knees fully extended Palms and hands can rest flat on the floor 1
Palms and hands cannot rest flat on the floor 0

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