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Neonatal lupus: Clinical spectrum, biomarkers, pathogenesis, and approach to treatment

Introduction Fetal exposure to maternal autoantibodies transported across the human placenta via FcγRn can result in a spectrum of organ injury: transient if the tissue has regenerative capacity and permanent if this capacity is absent or limited. An important example of passively acquired autoimmunity in a fetus and neonate is that of neonatal lupus (NL) given the concordance between circulating maternal antibody in the fetus and…

Fertility and pregnancy in systemic lupus erythematosus

Systemic lupus erythematosus—A manual Systemic lupus erythematosus (SLE) is a disease of reproductive aged women; as a result, family planning issues such as fertility and pregnancy are an important component of the management of SLE patients. For many years, women who had systemic lupus erythematosus were counseled against becoming pregnant. This directive was informed by the belief that the hormonal and immunologic changes of pregnancy would…

Systemic lupus erythematosus and the eye

Introduction Ocular involvement is common in systemic lupus erythematosus (SLE), with around one-third of patients being affected to some extent. Ophthalmic features are protean, with the most common complication is dry eye syndrome, which, though it may cause significant discomfort, is usually responsive to treatment and rarely affects vision. Sight-threatening complications of SLE are uncommon and include retinal vaso-occlusive disease, scleritis, and optic neuropathy ( Table 51.1…

Overlap syndromes

Introduction Overlap syndromes are conditions in which patients have concurrent clinical manifestations of multiple distinct immune diseases. A relatively common example of an overlap syndrome is mixed connective tissue disease (MCTD), in which antismall nuclear ribonucleoprotein (snRNP, or RNP) antibodies occur along with manifestations of at least two of the following: lupus, myositis, rheumatoid arthritis, and/or scleroderma. Patients with overlap syndromes need not meet classification criteria…

The nervous system in systemic lupus erythematosus

Introduction The immune dysregulation observed in lupus can damage every level of the nervous system ( Fig. 49.1 ). To complicate matters, neurological disease in lupus is not limited to direct immunological damage and can arise from comorbid disease, or as a complication of therapy. As a result, neurological disease in lupus is heterogeneous, complex, and poorly understood. “Neurolupus” is perhaps the simplest term to use to…

Malignancies in systemic lupus erythematosus

Introduction Many studies have shown unique cancer profiles for patients with autoimmune conditions such as rheumatoid arthritis (RA), Sjögren's syndrome, scleroderma, and systemic lupus erythematosus (SLE). Much of the data on cancer in SLE in general, and hematologic cancers specifically, comes from a very large (over 16,000 patients) multi-center international study of clinical SLE cohorts (which we will refer to later in this article). Although the…

Systemic lupus erythematosus and infections

Introduction Infections represent a significant contributor to morbidity and mortality among patients with systemic lupus erythematosus (SLE). Inherent dysfunction in innate and adaptive immune responses as well as chronic immunosuppressive treatment begets vulnerability to acute and chronic infections. Patients with SLE may also face separate challenges related to accrued end-organ damage, functional hyposplenism, and reticuloendothelial system flaws. These further hamper the ability to defend against infections.…

Gastrointestinal, hepatic, and pancreatic disorders in systemic lupus erythematosus

Introduction Gastrointestinal (GI) manifestations of systemic lupus erythematosus (SLE) are protean ( Table 46.1 ). Clinical features are noncharacteristic and must be distinguished from infective, thrombotic, therapy-related, and nonSLE causes. Appropriate imaging investigations, endoscopic procedures, and biopsies are indicated. Early recognition and intervention help reduce mortality and morbidity. Table 46.1 Gastrointestinal and hepatic manifestations of lupus. Oral cavity Oral ulceration Mucosal discoid lupus Sicca symptoms Chronic periodontitis…

The lung in systemic lupus erythematosus

Introduction Systemic lupus erythematosus (SLE) is an autoimmune disorder that can affect virtually every organ of the body, including the lung. Depending on the sensitivity of the tools used to detect disease and the populations studied, the incidence of pulmonary involvement in SLE varies. However, it is likely that 60% and higher of SLE patients experience lung involvement at some stage in the course of their…

Cardiovascular disease in systemic lupus erythematosus: an update

Burden of cardiovascular disease in lupus Despite therapeutic advances, cardiovascular disease (CVD) remains a major cause of morbidity and mortality in systemic lupus erythematosus (SLE, lupus). Although a nationwide population-based study from 1968–2013 found improving mortality in SLE, with a consistent downtrend since 1999, SLE-related mortality continues to remain high compared to non-SLE-related mortality. Furthermore, significant disparities in mortality based on sex, race/ethnicity, age, and geographic…

The pathology of lupus nephritis

Introduction The kidney, an “innocent bystander” in the pathogenesis of SLE, nevertheless bears the brunt of the morbidity and mortality of the disease. Indeed the majority of patients with lupus eventually develop some degree of renal involvement, which in some cases precedes (occasionally by years) the diagnosis of SLE. This clinically oriented chapter will focus on the varied expressions of SLE associated renal disease, their classification…

The clinical evaluation of kidney disease in systemic lupus erythematosus

Introduction Kidney disease is common in patients with systemic lupus erythematosus (SLE). This is most often due to lupus nephritis (LN). LN is one of the major causes of morbidity and mortality in SLE patients and is associated with poorer outcomes than in those patients with no kidney involvement. This poor prognosis is explained only in part by the risk of chronic kidney disease (CKD) and…

Cutaneous lupus erythematosus

Epidemiology Cutaneous manifestations occur in approximately 75% of patients with systemic lupus erythematosus (SLE) during the course of the disease and are the first sign in about 25% of patients. Epidemiological data of the different subtypes of cutaneous lupus erythematosus (CLE) have rarely been investigated, as most studies rather evaluate the incidence of SLE. In 2007, a study from Stockholm County, Sweden, reported that subacute cutaneous…

The musculoskeletal system in SLE

Along with cutaneous disease, musculoskeletal manifestations predominate in SLE with large series observing more than a 90% cumulative involvement, whereas some musculoskeletal complications, such as myositis and arthritis, reflect disease activity, others, such as osteonecrosis and osteoporosis, are typically adverse effects of treatment. In this chapter, we review common musculoskeletal manifestations that affect patients with SLE. Arthritis Clinical trials confirmed the high prevalence of arthritis in…

Constitutional symptoms and fatigue in systemic lupus erythematosus

Introduction Constitutional symptoms are very common in systemic lupus erythematosus (SLE) but are rather nonspecific; therefore, they are not taken into account in the classification criteria for SLE. Fatigue is the most common constitutional symptom associated with SLE, affecting up to 90% of patients. Other constitutional symptoms such as fever, anorexia, lymphadenopathy, and splenomegaly are less common. The presence of constitutional symptoms may reflect ongoing active…

Pathogenesis of tissue injury in the brain in patients with systemic lupus erythematosus

The brain and nervous system are important targets of immunological injury in people with systemic lupus erythematosus. The nature of this brain injury is complex and all levels of the neuroaxis can be affected, including the brain parenchyma, spinal cord, the cerebral vasculature, and the peripheral nervous system. Compared to other organ system, our mechanistic understanding of brain disease in lupus remains modest, and represents a…

The mechanism of skin damage

Introduction Lupus erythematosus (LE) comprises a broad spectrum of chronic inflammatory autoimmune disorders that display many diverse symptoms. Localized cutaneous LE (CLE) and severe systemic LE (SLE) represent the two ends of the spectrum of LE. The various cutaneous manifestations of LE are divided into LE-specific and LE-nonspecific skin disease based on histopathological findings. The underlying cause of lupus like lesion is unknown but it is…

Mechanisms of vascular damage in systemic lupus erythematosus

Epidemiology of vascular damage in systemic lupus erythematosus Accelerated atherosclerosis leading to cardiovascular (CV) events is a well-established cause of morbidity and mortality in patients with systemic lupus erythematosus (SLE). A bimodal mortality pattern in SLE was recognized in the 1970s, and these observations of enhanced mortality late in the course of SLE disease due primarily to CV disease (CVD) have been replicated in several other…

Mechanisms of renal damage in systemic lupus erythematosus

Introduction Renal involvement of systemic lupus erythematosus (SLE) was first reported by W. Osler in 1895. “Wire loop” glomeruli, the pathological lesions that are characteristic of lupus nephritis (LN) were described by Baehr et al in 1935. With the advent of immunofluorescence microscopy, Ig deposits in the kidneys of LN were recognized and complement deposition was demonstrated. Subsequently Ig was eluted from diseased kidney. The eluted Ig…

Genes and genetics of murine systemic lupus erythematosus

Introduction Systemic lupus erythematosus (SLE) is inherited as a multifactorial trait with genetic predisposition playing a major role. Consequently, there has been considerable interest in defining the genetics of this disease. Progress in this area has advanced tremendously over the past few years, resulting in the identification of more than 100 genes associated with lupus risk variants. This effort has been greatly facilitated by studies in…