An idiopathic disorder characterized by recurrent inflammation of cartilaginous structures. Cardinal manifestations include ear and nose involvement with floppy ear and saddlenose deformities, inflammation and collapse of tracheal and bronchial cartilaginous rings, and asymmetric episodic nondeforming polyarthritis. Other features can include scleritis, conjunctivitis, iritis, keratitis, aortic regurgitation, glomerulonephritis, and other features of systemic vasculitis. Onset is frequently abrupt, with the appearance of 1–2 sites of cartilaginous inflammation. Diagnosis is made clinically and may be confirmed by biopsy of affected cartilage.
TREATMENT Relapsing Polychondritis
Glucocorticoids (prednisone 40–60 mg/d with subsequent taper) may suppress acute features and reduce the severity/frequency of recurrences. Cytotoxic and other immunosuppressive agents should be reserved for unresponsive disease or for pts who require high glucocorticoid doses. When airway obstruction is severe, tracheostomy may be required.
POLYMYALGIA RHEUMATICA (PMR)
Clinical syndrome characterized by aching and morning stiffness in the shoulder girdle, hip girdle, or neck for >1 month, elevated ESR, and rapid response to low-dose prednisone (10–20 mg qd). Rarely occurs before age 50; more common in women. PMR can occur in association with giant cell arteritis, which requires treatment with higher doses of prednisone. Evaluation should include a careful history to elicit symptoms suggestive of giant cell arteritis (Chap. 165); ESR; laboratory test results to rule out other processes usually include RF, ANA, CBC, CPK, serum protein electrophoresis; and renal, hepatic, and thyroid function tests.
Pts rapidly improve on prednisone, 10–20 mg daily, but may require treatment over months to years.
NEUROPATHIC JOINT DISEASE
Also known as Charcot’s joint, this is a severe destructive arthropathy that occurs in joints deprived of pain and position sense; may occur in diabetes mellitus, tabes dorsalis, syringomyelia, amyloidosis, spinal cord, or peripheral nerve injury. Distribution depends on the underlying joint disease. Joint effusions are usually noninflammatory but can be hemorrhagic. Radiographs can reveal either bone resorption or new bone formation with bone dislocation and fragmentation.
TREATMENT Neuropathic Joint Disease
Stabilization of joint; surgical fusion may improve function.
Syndrome consisting of periosteal new bone formation, digital clubbing, and arthritis. Most commonly seen in association with lung carcinoma but, also occurs with selected chronic lung or liver disease; congenital heart, lung, or liver disease in children; and idiopathic and familial forms. Symptoms include burning and aching pain most pronounced in distal extremities. Radiographs show periosteal thickening with new bone formation of distal ends of long bones.
TREATMENT Hypertrophic Osteoarthropathy
Identify and treat associated disorder; aspirin, NSAIDs, other analgesics, vagotomy, or percutaneous nerve block may help to relieve symptoms.