Rheumatology

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

Definition Vasculitis is a clinicopathologic process characterized by inflammation of and damage to blood vessels. The vessel lumen is usually compromised, and this is associated with ischemia of the tissues supplied by the involved vessel. A broad and heterogeneous group of syndromes may result from this process, since any type, size, and location of blood […]

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

Wegener’s Granulomatosis Definition Wegener’s granulomatosis is a distinct clinicopathologic entity characterized by granulomatous vasculitis of the upper and lower respiratory tracts together with glomerulonephritis. In addition, variable degrees of disseminated vasculitis involving both small arteries and veins may occur. Incidence and Prevalence Wegener’s granulomatosis is an uncommon disease with an estimated prevalence of 3 per […]

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

Churg-Strauss Syndrome Definition Churg-Strauss syndrome, also referred to as allergic angiitis and granulomatosis, was described in 1951 by Churg and Strauss and is characterized by asthma, peripheral and tissue eosinophilia, extravascular granuloma formation, and vasculitis of multiple organ systems. Incidence and Prevalence Churg-Strauss syndrome is an uncommon disease with an estimated annual incidence of 1-3 […]

The Vasculitis Syndromes (Disorders of Immune-Mediated Injury) (Rheumatology) Part 4

Takayasu’s Arteritis Definition Takayasu’s arteritis is an inflammatory and stenotic disease of medium- and large-sized arteries characterized by a strong predilection for the aortic arch and its branches. For this reason, it is often referred to as the aortic arch syndrome. Incidence and Prevalence Takayasu’s arteritis is an uncommon disease with an estimated annual incidence […]

The Vasculitis Syndromes (Disorders of Immune-Mediated Injury) (Rheumatology) Part 5

Behget’s syndrome Behget’s syndrome is a clinicopathologic entity characterized by recurrent episodes of oral and genital ulcers, iritis, and cutaneous lesions. The underlying pathologic process is a leukocytoclastic venulitis, although vessels of any size and in any organ can be involved. This disorder is described in detail in Chap. 11. Isolated vasculitis of The Central […]

Behçet’s Syndrome (Disorders of Immune-Mediated Injury) (Rheumatology)

Definition, Incidence, and Prevalence Behget’s syndrome is a multisystem disorder presenting with recurrent oral and genital ulcerations as well as ocular involvement. The diagnosis is clinical and based on internationally agreed diagnostic criteria (Table 11-1). The syndrome affects young males and females from the Mediterranean region, the Middle East, and the Far East, suggesting a […]

Relapsing Polychondritis (Disorders of Immune-Mediated Injury) (Rheumatology)

Relapsing polychondritis is an uncommon inflammatory disorder of unknown cause characterized by an episodic and generally progressive course affecting predominantly the cartilage of the ears, nose, and laryngotracheo-bronchial tree. Other manifestations include scleritis, neu-rosensory hearing loss, polyarthritis, cardiac abnormalities, skin lesions, and glomerulonephritis. The peak age of onset is between 40 and 50 years, but […]

Sarcoidosis (Disorders of Immune-Mediated Injury) (Rheumatology) Part 1

Definition Sarcoidosis is an inflammatory disease characterized by the presence of noncaseating granulomas. The disease is often multisystem and requires the presence of involvement in two or more organs for a specific diagnosis. The finding of granulomas is not specific for sarcoidosis, and other conditions known to cause granulomas must be ruled out. These conditions […]

Sarcoidosis (Disorders of Immune-Mediated Injury) (Rheumatology) Part 2

Eye The frequency of ocular manifestations for sarcoidosis varies depending on race. In Japan, >70% of sarcoidosis patients develop ocular disease, while in the United States only 30% have eye disease, with problems more common in blacks than whites. Although the most common manifestation is an anterior uveitis, over a quarter of patients will have […]

Sarcoidosis (Disorders of Immune-Mediated Injury) (Rheumatology) Part 3

Prognosis The risk of death or loss of organ function remains low in sarcoidosis. Poor outcomes usually occur in patients who present with advanced disease in whom treatment seems to have little impact. In these cases, irreversible fibrotic changes have frequently occurred. For the majority of patients, initial presentation occurs during the granulomatous phase of […]

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