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Endoscopic scores in inflammatory bowel disease


Abbreviations

CD

Crohn’s disease

CDAI

the Crohn’s Disease Activity Index

CDEIS

the Crohn’s Disease Endoscopic Index of Severity

CRP

C-reactive protein

DAI

the Diseases Activity Index

GI

gastrointestinal

HBI

the Harvey–Bradshaw Index

IBD

inflammatory bowel disease

ICA

ileocolonic anastomosis

ICR

ileocolonic resection

IPAA

ileal pouch-anal anastomosis

MES

the Mayo Endoscopic Subscore

MH

mucosal healing

MMES

the modified Mayo Endoscopic score

MRE

magnetic resonance enterography

PDAI

the Pouchitis Disease Activity Index

RS

the Rutgeerts Score

SES-CD

the simple endoscopic score for Crohn’s disease

STRIDE

selecting therapeutic targets in inflammatory bowel disease

UC

ulcerative colitis

UCEIS

the Ulcerative Colitis Endoscopic Index of Severity

UCCIS

the Ulcerative Colitis Colonoscopic Index of Severity

Introduction

Endoscopy is a standard modality for diagnosis, differential diagnosis, assessment of disease activity, and response to treatment in Crohn’s disease (CD) and ulcerative colitis (UC). Endoscopy provides the most reliable measurement of mucosal inflammation. The goal of management of inflammatory bowel disease (IBD) is constantly evolving. Earlier clinical trials have used clinical parameters to measure disease activity before and after therapeutic intervention. Recent studies have shown that endoscopic healing documented by high-definition colonoscopy is associated with less complication and better long-term outcomes . The selecting therapeutic targets in IBD (STRIDE) initiated by the he International Organization for the Study of IBD proposed target goals for both CD and UC. The treat-to-target concept is being accepted in clinical practice. The targets are clinical, biological, and histologic. Endoscopy targets, that is, endoscopic mucosal healing (MH), are established with objective documentation of response to medical or surgical treatment. Endoscopic MH were defined as the absence of ulceration for CD or Mayo Endoscopic Subscore (MES) of 0 or 1 for UC . Multiple endoscopic scores have been developed for CD, UC, and pouchitis, mainly based on features of white-light endoscopy. In this chapter, we present some of the most commonly used endoscopic scores with a wide range of illustrative images.

Components of endoscopic assessment of mucosal inflammation

The main components of endoscopic features shared by CD and UC are erythema, edema, loss of vascular pattern, granularity, nodularity, friability, spontaneous bleeding, erosions, ulcers, and pseudopolyps ( Fig. 14.1 ). These inflammation conditions are often accompanied by the presence of mucopurulent exudates on the top of the erosion or ulcers or nonulcerated inflamed surface ( Fig. 14.2 ).

Figure 14.1, Common patterns of inflammation of Crohn’s disease and ulcerative colitis: (A) erythema, (B) edema, (C) loss of vascular pattern, (D) nodularity, (E) friability, (F) spontaneous bleeding, (G) erosions, (H) ulcers, and (I) pseudopolyps.

Figure 14.2, Additional patterns of mucosal inflammation in Crohn’s disease: (A) exudates in the absence of mucosal inflammation and (B–D) exudates cover ulcers and exudates with histology.

There are endoscopic modifiers for CD, including cobblestoning and strictures. These modifiers can make an accurate measurement of disease activity complex. Cobblestoning is defined as serpiginous and linear ulcers intervened with nodular mucosa, which is commonly seen in the distal lymphoid tissue-predominant terminal ileum. This pattern of disease process gives rise to deep linear ulcer “cracks” in between the areas of inflamed or normal tissue with an appearance of “stones.” The width of the ulcer cracks and the size of nodular mucosa vary ( Fig. 14.3 ).

Figure 14.3, Spectrum of cobblestoning and nodular mucosa in Crohn’s disease: (A and B) classic cobblestoning with networks of thin ulcers and nodular mucosa and (C and D) nodular mucosa with networks of deep and wider ulcers in between.

Another endoscopic modifier is the presence of stricture. Strictures can be observed in both CD and UC. Endoscopic appearance of strictures can be short or long, mild or severe, inflammatory or fibrotic, and ulcerated or nonulcerated. Nonulcerated strictures can be a part of mucosal or transmural healing or serosa- or mesentery-predominant transmural disease processwith normal overlying mucosa ( Figs. 14.4 and 14.5C and D ). On the other hand, the presence of ulcers on the anastomosis does not necessarily indicate active CD or UC. Rather they may result from surgery-associated ischemia or the use of nonsteroidal antiinflammatory drugs ( Fig. 14.6 ).

Figure 14.4, Nonulcerated strictures in Crohn’ disease resulting from disease process per se or effective medical therapy with tissue healing (A–D).

Figure 14.5, Forms of endoscopic mucosal healing: (A) mucosal scars, (B)

Figure 14.6, Inflammatory versus ischemic strictures in Crohn’s disease patients with ileocolonic resection and anastomosis: (A) ulcerated stricture can be due to ischemia with a normal proximal segment of bowel, (B and C) inflammatory ulcerated strictures, and (D) inflammatory nodular stricture.

Crohn’s disease

The disease activity of CD has been measured with clinical, endoscopic, laboratory, and histologic features. Clinical and endoscopic disease activity instruments are routinely used in clinical trials and are increasingly used in clinical practices.

Instruments for clinical disease activity

The 1100-point Crohn's Disease Activity Index (CDAI) was proposed and has been routinely used in clinical trials . The items listed in the CDAI consist of the average number of liquid or soft stools per day over 7 days—using diphenoxylate or loperamide for diarrhea, average abdominal pain rating over 7 days; general well-being each day over 7 days; arthritis or arthralgia, iritis or uveitis, erythema nodosum, pyoderma gangrenosum, or aphthous stomatitis, anal fissure, fistula, or abscess, other fistula, temperature more than 100°F (37.8°C) in the last week; and finding of abdominal mass. CDAI has been used for quantification of clinical disease activity : asymptomatic remission (CDAI 0–149), mild disease (CDAI 150–220), moderate-to-severe disease (CDAI 221–450), and severely active to fulminant disease (CDAI 451–1100). Patients requiring corticosteroids to remain asymptomatic should not be considered as being in remission. Rather, they are referred as being “steroid-dependent.”

The Harvey–Bradshaw Index (HBI) of CD activity is a simplified clinical instrument . Components of HBI consist of patient sense of general well-being (0–4) and abdominal pain (0–3) in the last 24 hours; number of liquid stools in last 24 hours, abdominal mass (0–4); and complications with arthralgias, uveitis, erythema nodosum, aphthous ulcers, pyoderma gangrenosum, anal fissures, newly discovered fistulae, or abscesses (0–8). An HBI score of <5 correlates with clinical remission.

The Crohn’s Disease Endoscopic Index of Severity

Recently, the use of endoscopic instruments as outcome measures for clinical research has been more emphasized, as the endoscopic assessment is more objective than symptom-based indices. The most commonly used and validated endoscopic indices are the 44-point Crohn's Disease Endoscopic Index of Severity (CDEIS) , the Simple Endoscopic Score for Crohn's Disease (SES-CD) , and the Rutgeerts Score (RS) for postoperative recurrence in the neo-terminal ileum after ileocolonic resection and anastomosis . These indices have been mainly applied for clinical trials. However, components of the indices are used for the diagnosis, differential diagnosis, disease monitoring, and assessment of response to therapy in clinical practice.

The first described CD endoscopic score is the 44-point CDEIS developed by Mary and Modigliani in 1989. Despite its correlation with clinical indices , this score involves a series of labor-intensive calculations. To calculate CDEIS, five segments of the intestine are assessed: the rectum, sigmoid and left colon, traverse colon, right colon, and ileum ( Table 14.1 ). The definition of severity is based on the size and depth of ulceration, that is, superficial ulceration ( Fig. 14.7 ) or deep ulceration ( Figs. 14.8 and 14.9 ; Table 14.2 ). The aphthoid lesion, though mentioned in the instrument and evaluated during its initial development, is not a component for the final calculation of CDEIS ( Fig. 14.10 ). In each colon segment CDEIS is calculated based on the presence of deep or superficial ulceration, the percentage of ulcerated surface; and the percentage of surface involved by CD are measured on a 10-cm visual analog scale ( Table 14.1 ). In addition, the presence of ulcerated and nonulcerated stenoses is assessed.

Table 14.1
Crohn’s Disease Endoscopic Index of Severity (CDEIS) .
Rectum Sigmoid and left colon Transverse colon Right colon Ileum Total
Deep ulceration quote 12 if present in the segment, 0 if absent Total 1
Superficial ulceration quote 6 if present in the segment, 0 if absent Total 2
Surface involved by the disease measured in cm a Total 3
Ulcerated surface measured in cm a Total 4
Total 1+Total 2+Total 3+Total 4=Total A. Number ( n ) of segments totally or partially explored (1–5)= n . Total A divided by n =Total B. Quote 3 if ulcerated stenosis anywhere, 0 if not=C. Quote 3 if nonulcerated stenosis anywhere, o if not=D. Total B+C+D=CDEIS.

a For partially explored segments and for the ileum, the 10 cm linear scale represents the surface effectively explored.

Figure 14.7, Superficial ulcers as a component in the CDEIS-

Figure 14.8, Deep ulceration as a component of the CDEIS: (A–D) deep ulcers in the terminal ileum. CDEIS , the Crohn’s Disease Endoscopic Index of Severity.

Figure 14.9, Deep ulceration as a component of CDEIS: (A) large ulcers in the terminal ileum, (B and C) large ulcers in the colon, and (D) ulcers in the rectum in a patient with colorectal anastomosis. CDEIS , the Crohn’s Disease Dndoscopic Index of Severity.

Table 14.2
Further definitions of Crohn’s Disease Endoscopic Index of Severity (CDEIS) .
Lesions Definitions
Aphthoid ulceration Tiny (2–3 mm), raised or flat red lesion with a white center
Superficial ulceration Ulceration which was neither aphthoid nor deep
Deep ulceration Only frankly deep ulceration

Figure 14.10, Various forms of aphthous ulceration in Crohn’s disease. Of note, the presence of aphthous ulcers is not the component of CDEIS but is a component for SES-CD. Aphthous ulceration was defined as tiny (2–3 mm in CDEIS or 0.1–0.5 mm in SES-CD), raised or flat red lesion with a white center: (A–C) aphthous ulcers or erosions in the terminal ileum and (D) aphthous ulcers or erosions in the colon with edematous adjacent mucosa. CDEIS , the Crohn’s Disease Endoscopic Index of Severity; SES-CD , the SEndoscopy Score of Crohn’s Disease.

The calculation of CDEIS is illustrated in Fig. 14.11A and B , corresponding to Tables 14.1 and 14.2 .

Figure 14.11, (A) Illustration of the calculation of CDEIS in a patient with inflammatory and fibrostenotic ileocolonic CD: (a) distal 5-cm rectum with deep ulcers, (b) a deeply ulcerated 6-cm-long stricture at the sigmoid colon with a 10-cm-long disease segment, (c) normal transverse, (d) an 8-cm-long deeply ulcerated area with stricture at the ascending colon with additional 2-cm-long inflamed area in the surrounding mucosa, and (e) a 5-cm-long area with superficial ulcers in the terminal ileum. (B) Calculation of CDEIS in the patient with inflammatory and fibrostenotic ileocolonic CD. CD , Crohn’s disease; CDEIS , the Crohn’s Disease Endoscopic Index of Severity.

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