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 impairment due to vasculitis; frequent arthritis, muscle and skin damage. The onset of fibrosing alveolitis is a response to immunocomplexes deposited in the pulmonary capillaries. The treatment is corticotherapy.


 * Rheumatoid arthritis
 * Intersticial 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 impairment in 50 to 60%: most often pleurisy, ILD, rarely acute pneumonia;
 * X-RAY: reticulonodular shadows with max. impairment 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)
 * ILD in up to 80% of patients


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

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 * Chronic lung disease • Interstitial lung disease
 * Rheumatoid arthritis • Systemic lupus erythematosus • Sclerodermy • Sjorgen's syndrome