Polychondritis, relapsing

Relapsing polychondritis is a systemic inflammatory disease of cartilage. At the time of first presentation, a red painful area of chondritis is often misdiagnosed as a cellulitis. Common areas of involvement include the outer ear and nose. 

However, the trachea is also made of cartilage and can be involved with repetitive cycles of inflammation, ulceration, and scarring that may ultimately present with tracheal stenosis. The two CTDs most often associated with tracheal stenosis include relapsing polychondritis and necrotizing granulomatous vasculitis. Stenosis in both diseases is most common in the subglottic space. 

In summary, the connective tissue disorders frequently involve the lung with a variety of disorders that impact clinical symptoms and outcome. Knowledge of these disorders will assist in optimal clinical outcomes. The impact of the pulmonary specialist is more appreciated when clinical care is provided in a comprehensive approach. Therefore, successful centers that deal with the CTDs foster collaborative care for these complicated disorders. 

Sahn SA. The pathophysiology of pleural effusions. Annu Rev Med 1990 41 7-13. Lillington GA, Carr DT, Ma3fne JG. Rheumatoid pleurisy with effusion. Arch Intern Med 1971 128(5) 764-768. 

Jones F, Blodgett R. Empyema in iheumatoid pleuropulmonary disease. Ann Intern 

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AOSD, adult onset Still disease AS, ankylosing spondylitis CA, crystal-induced arthritis ERA, enthesitis-related arthritis JA, juvenile arthritis PA, psoriatic arthritis RA, rheumatoid arthritis SE, synovium explants SLE, systemic lupus erythematosus SPCIA, solid phase 2 site chemiluminescent immunometric assay RP, relapsing polychondritis. 

Stabler T, Piette JC, Chevalier X, Marini-Portugal A, Kraus VB. Serum cytokine profiles in relapsing polychondritis suggest monocyte/macrophage activation. Arthritis Rheum 2004 50(11) 3663—367. 

Relapsing polychondritis Progressive systemic sclerosis Sarcoid angiitis... 

Relapsing polychondritis may be complicated by a generalized encephalopathy, stroke-like episodes and ischemic optic neuropathy as a result of systemic vasculitis (Stewart et al 1988 Hsu et al 2006). 

Stewart SS, Ashizawa T, Dudley AW et al. (1988). Cerebral vasculitis in relapsing polychondritis. Neurology 38 150-152 Stockhammer G, Felber SR, Zelger B et al (1993). Sneddon s syndrome diagnosis by skin biopsy and MRI in 17 patients. Stroke 24 685-690... 

Inflammatory bowel disease Relapsing polychondritis Rheumatoid arthritis Systemic lupus erythematosus Infectious Herpes zoster Lyme disease Syphilis Other... 

Studies of patients with borderline leprosy have suggested that dapsone has mild immunosuppressive effects (SEDA-18, 30). Thus, it has been given with some success to patients with dermatitis herpetiformis, subcorneal pustular dermatosis, bullous dermatoses, relapsing polychondritis, thrombocytopenic purpura (3), giant cell arteritis (4), rheumatoid arthritis, and systemic lupus erythematosus (5). 

Koenig AS, Abruzzo JL. Leflnnomide induced fevers, thrombocytosis, and lenkocytosis in a patient with relapsing polychondritis. J Rhenmatol 2002 29(1) 192. ... 

This feature is associated with the following diseases and conditions COPD, relapsing polychondritis, history of prolonged intubation, or prior radiotherapy. 

Cardiovascular A 51-year-old woman with relapsing polychondritis presented with acute myocarditis 2 weeks after a second course of adalimumab. Cardiac arrest followed within a few hours with autopsy showing necrotising eosinophilic myocarditis. This appears to be the first report of a TNFa antagonist associated with this condition [74 ]. 

Adamson R, Yazici Y, Katz ES, Greisman SG, Steiger D. Fatal acute necrotizing eosinophilic myocarditis temporally related to use of adaH-mumab in a patient with relapsing polychondritis. J CHn Rheumatol 2013 19(7) 386-9. 

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