Pulmonary manifestations in systemic connective tissue diseases

> náhled|vpravo|250px|Postižené orgány při lupusu náhled|vpravo|250px|Revmatoidní artritida ruky Systemic connective tissue diseases are autoimmune diseases with multiorgan involvement due to vasculitis; frequent arthritis, muscle and skin damage. The onset of fibrotic alveolitis is a response to immunocomplexes deposited in the pulmonary capillaries. The treatment is corticotherapy.


 * Rheumatoid arthritis
 * Interstitial damage in 1.5 to 4.5%;
 * clinically and histologically identical to KFA;
 * prognosis: unfavorable in case of pulmonary changes;
 * therapy: glucocorticoids + immunosuppressants.


 * Systemic lupus erythematosus
 * Pulmonary involvement in 50 to 60%: most often pleurisy, ILD, rarely acute pneumonia;
 * X-RAY: reticulonodular shadows with max. involvement of the lower lung fields;
 * therapy: corticoids + penicillamine/cyclophosphamide;
 * survival 10 to 14 years (cause of death renal failure, endarteritis or secondary pneumonia).


 * Scleroderma (progressive systemic sclerosis)
 * IPP in up to 80% of patients


 * Polymysitis, dermatomyositis
 * Sjogren's syndrome
 * Bechterew's disease
 * Crohn's disease

Související články

 * Chronická onemocnění plic • Intersticiální plicní procesy
 * Revmatoidní artritida • Systémový lupus erythematodes • Sklerodermie • Sjögrenův syndrom