Rheumatology

Acute Rheumatic Fever (Disorders of Immune-Mediated Injury) Part 1

Acute rheumatic fever (ARF) is a multisystem disease resulting from an autoimmune reaction to infection with group A streptococci. Although many parts of the body may be affected, almost all of the manifestations resolve completely. The exception is cardiac valvular damage [rheumatic heart disease (RHD)], which may persist after the other features have disappeared. * […]

Acute Rheumatic Fever (Disorders of Immune-Mediated Injury) Part 2

Treatment: Acute Rheumatic Fever Patients with possible ARF should be followed closely to ensure that the diagnosis is confirmed, treatment of heart failure and other symptoms is undertaken, and preventive measures including commencement of secondary prophylaxis, inclusion on an ARF registry, and health education are commenced. Echocardiography should be performed on all possible cases to […]

Systemic Sclerosis (Scleroderma) and Related Disorders (Disorders of Immune-Mediated Injury) (Rheumatology) Part 1

Definition Systemic sclerosis (SSc) is a chronic systemic disorder of unknown etiology. SSc is characterized by thickening of the skin (scleroderma) and distinctive involvement of multiple internal organs, most notably the lungs, gastrointestinal tract, heart, and kidneys. The early stage of the disease, associated with prominent inflammatory features, is followed by the development of widespread […]

Systemic Sclerosis (Scleroderma) and Related Disorders (Disorders of Immune-Mediated Injury) (Rheumatology) Part 2

Pathology The distinguishing pathologic hallmark of SSc is the combination of widespread obliterative vasculopathy of small arteries and arterioles and fibrosis in the skin and internal organs. Whereas in established SSc these lesions occur in the absence of inflammation, in relatively early-stage disease, perivascular cellular infiltrates may be detected in multiple organs prior to the […]

Systemic Sclerosis (Scleroderma) and Related Disorders (Disorders of Immune-Mediated Injury) (Rheumatology) Part 3

Pulmonary Features Pulmonary involvement can be documented in most patients with SSc and is now the leading cause of death. The two main types of significant pulmonary involvement are ILD and PAH; many patients develop some degree of both complications. Less frequent pulmonary manifestations include aspiration pneumonitis complicating gastroesophageal reflux, pulmonary hemorrhage due to endobronchial […]

Sjögren’s syndrome (Rheumatology) Part 1

Definition, Incidence, and Prevalence Sjögren’s syndrome is a chronic, slowly progressive autoimmune disease characterized by lymphocytic infiltration of the exocrine glands resulting in xerostomia and dry eyes. Approximately one-third of patients present with systemic manifestations; a small but significant number of patients may develop malignant lymphoma. The disease presents alone (primary Sjögren’s syndrome) or in […]

Sjögren’s syndrome (Rheumatology) Part 2

Treatment: Sjögren’s Syndrome Treatment of Sjögren’s syndrome is aimed at symptomatic relief and limiting the damaging local effects of chronic xerostomia and keratoconjunctivitis sicca by substituting or simulating the missing secretions (Fig. 8-1 ). To replace deficient tears, there are several readily available ophthalmic preparations (Tearisol; Liquifilm; 0.5% methylcellulose; Hypo Tears). If corneal ulcerations are […]

The Spondyloarthritides (Disorders of Immune-Mediated Injury) (Rheumatology) Part 1

The spondyloarthritides are a group of disorders that share certain clinical features and genetic associations. These disorders include ankylosing spondylitis, reactive arthritis, psoriatic arthritis and spondylitis, enteropathic arthritis and spondylitis, juvenile-onset spondyloarthritis, and undifferentiated spondyloarthritis. The similarities in clinical manifestations and genetic predisposition suggest that these disorders share pathogenic mechanisms. Ankylosing spondylitis Ankylosing spondylitis (AS) […]

The Spondyloarthritides (Disorders of Immune-Mediated Injury) (Rheumatology) Part 2

Treatment: Ankylosing spondylitis Any management of AS should include an exercise program designed to maintain posture and range of motion. Until recently, nonsteroidal anti-inflammatory drugs (NSAIDs) have been the mainstay of pharmacologic therapy for AS.These agents reduce pain and tenderness and increase mobility in many patients with AS. Moreover, in a recent 2-year randomized controlled […]

The Spondyloarthritides (Disorders of Immune-Mediated Injury) (Rheumatology) Part 3

Epidemiology Estimates of the prevalence of PsA among individuals with psoriasis range from 5 to 30%. In Caucasian populations, psoriasis is estimated to have a prevalence of 1-3%. Psoriasis and PsA are less common in other races in the absence of HIV infection. First-degree relatives of PsA patients have an elevated risk for psoriasis, for […]

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