Systemic Manifestations

Joint involvement is the most common manifestation outside the head and neck. Arthritis is present in 35% to 40% of patients at the time of diagnosis and occurs in 50% to 85% during the course of RP (24). Joint involvement can consist of arthralgias or active synovitis. Peripheral arthritis involves the small and large joints and can be mono-, oligo-, or polyarticular. It is usually asymmetric but can be symmetric, resembling rheumatoid arthritis. Unlike rheumatoid arthritis, however, the arthritis of RP is seronegative, nonerosive, nondeforming, and associated with a noninflammatory synovial fluid. The joints most commonly involved are the metacarpophalangeal joints, proximal interpha-langeal joints, wrists, and knees. Up to 25% can have involvement of the thoracic cage, including the sternoclavicular, costochondral, and manubriosternal joints. Joint symptoms typically resolve spontaneously or with therapy within days to weeks, but usually recur at variable intervals. Joint involvement is a poor prognostic sign, as it is usually associated with more widespread disease.

Cardiovascular involvement occurs in 5% to 10% of patients with RP. Aortic and/or mitral valvular insufficiency is the most common manifestation, occurring in 2% to 8% of patients, particularly males. It can occur within months to years after diagnosis and usually progresses insidiously (25). Therefore, any new or changing murmur must be investigated. The valvular insufficiency results from aortic root dilatation, aortic or mitral valvular annulus dilatation, or destruction of the valvular cusps, frequently with myxoid degeneration in the center of the valve (26). Less common manifestations include pericarditis, myocarditis, and variable degrees of heart block. Vasculitis involving small, medium, and large arteries can occur in up to 15% of patients, manifesting as leukocytoclastic vasculitis with palpable purpura, myocardial infarction from coronary arteritis, or thoracic/abdominal/subclavian aneurysms which can rupture (Fig. 4) (27).

Various skin manifestations can be a presenting symptom in 12% to 16% of patients and occur in up to 36% of patients during the course of the disease. None of the skin findings are pathognomonic, but can include mucosal ulcers, septal panniculitis with nodules, palpable purpura, ulcerations due to leukocytoclastic vasculitis, and superficial thrombophlebitis from thrombosis (28). Several other skin lesions have also been reported

Polyarteritis Chondtritis
FIGURE 4 (Left) Tomogram of the trachea showing sub-glottic edema and tracheal narrowing in a patient with relapsing polychondritis. (Right) Aortogram demonstrates a large aneurysm of the ascending aorta in a patient with relapsing poly-chondritis. Source: American College of Rheumatology.

in RP patients. Patients with an associated myelodysplastic syndrome are particularly likely to have skin lesions. The combination of Behget's-like features with RP has been called the "MAGIC syndrome" (mouth and genital ulcers with inflamed cartilage) (29).

Other systemic manifestations reported in RP patients include renal and neurologic diseases (30). Kidney disease occurs in 5% to 10% of patients and portends a worse prognosis. Up to 25% can have an abnormal urinalysis as evidenced by microscopic hematuria, proteinuria, or elevated creatinine. The most common renal biopsy findings are a mesangial glomerulonephritis with immune deposits and a segmental necrotizing glomerulo-nephritis with crescents. Tubulointerstitial disease and IgA nephropathy are also reported. Most renal disease in patients with RP is due to an associated vasculitis and not to RP itself. Similarly, neurologic manifestations in RP are usually due to a vasculitis. Neurologic disease may be acute or subacute and cause significant morbidity and mortality (31). The most common manifestation is a cranial neuropathy (cranial nerves II, VI, VII, VIII). Other manifestations include stroke syndromes, seizures, dementia, aseptic meningitis, and peripheral neuropathy.

Up to 30% of patients with RP will have a coexistent autoimmune, hematologic, or inflammatory disease which usually precedes RP by months to years. Importantly, many of the systemic manifestations listed above may be manifestations of the underlying associated disease and not directly due to RP.

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