Articles on Medical Diseases and Conditions

Entries for September, 2009

Wegener’s Granulomatosis (Granulomatous Vasculitis)

Wegener’s granulomatosis is characterized by granulomatous inflammation containing foci of vasculitis involving the nasopharynx plus crescentic glomerulonephritis (proliferative vascular lesions obliterating a focal area of Bowman’s capsule space). Laboratory test abnormalities include substantial hematuria and proteinuria, and reflect a culture-negative ongoing inflammatory process. The WBC count and ESR are usually elevated when the disease is […]

Allergic (Hypersensitivity) Vasculitis

The most common and well-known entity in this category is Henoch-Schonlein (H-S) purpura. The primary abnormality is necrotizing inflammation of small venules, characterized by infiltration of the vessel wall by segmented neutrophils accompanied by destruction of some neutrophils with release of nuclear fragments (“leukocytoclastic vasculitis”). Immunohistologic methods can demonstrate IgA deposition in the vessel wall […]

Syndromes due Primarily to Autoimmune Vasculitis. Polyarteritis Nodosa.

Polyarteritis nodosa features inflammation of small and medium-sized arteries, most often in middle-aged males. Single organs or multiple systems may be involved, although usually the lungs are spared. The kidney is the organ most frequently involved (about 77% of cases), with hematuria the main sign. Peripheral nerves are affected almost as frequently, either with clinical […]

Sjogren’s Syndrome

The parotid gland disease formerly known as “Mikulicz’s syndrome” now is usually included with Sjogren’s Syndrome. Some reports indicate that Sjogren’s Syndrome affects 2%-5% of adults over age 55 and is second only to RA in frequency among the rheumatoid-collagen diseases. SSjogren’s Syndrome occurs in primary and secondary forms. Primary Sjogren’s Syndrome, sometimes called the […]

Mixed Connective Tissue Disease (MCT)

As the name implies, MCT disease contains elements of several rheumatoid-collagen entities, including SLE, PSS, and dermatomyositis. Clinically, there is arthralgia or arthritis in 90%-95% of patients, Raynaud’s phenomenon in about 80%-90%, abnormal esophageal motility in about 70%, muscle pain in about 70%, and fever in about 30%. There is a low incidence of renal […]

Progressive Systemic Sclerosis (Scleroderma; PSS)

PSS leads to progressive dense connective tissue replacement in certain areas that normally contain only small amounts of loose collagen. These include the dermis of the skin, the submucosa of the esophagus or other parts of the GI tract, and the heart. The lungs frequently develop a slowly progressive type of diffuse fibrosis, radiologically most […]

Systemic Lupus Erythematosus (SLE)

SLE features various combinations of facial skin rash, arthritis, nephritis, systemic symptoms such as fever and malaise, and inflammation of serous membranes such as the pericardium. The disease is by far more frequent in women, predominantly young or middle-aged adults. In one large series the mean age at diagnosis was 30 years, with a skewed […]

Collagen-Vascular Diseases

Collagen-vascular diseases are an ill-defined collection of syndromes that have certain points of similarity, among which the most striking are fibrinoid necrosis of collagenous tissue and involvement of various subdivisions of arteries by an inflammatory process. Some diseases emphasize one aspect and some the other. Because blood vessel inflammation and other abnormalities found in the […]

Acute Rheumatic Fever (ARF)

ARF is a disease that has a specific etiologic agent yet has some similarities to the rheumatoid-collagen-vascular group. The etiologic agent is the beta-hemolytic Lancefield group A Streptococcus. Apparent hypersensitivity or other effect of this organism causes connective tissue changes manifested by focal necrosis of collagen and the development of peculiar aggregates of histiocytes called […]

Seronegative Spondyloarthropathies

In these conditions arthritis is associated with inflammation that affects the spine and lumbosacral joints (ankylosing spondylitis), the urethra (Reiter’s syndrome), the skin (psoriasis), or the intestinal tract. These conditions were (and are) frequently referred to as “rheumatoid arthritis variants.” This name has been discarded by most rheumatologists because the diseases have relatively little in […]