Zeitschrift für Rheumatologie
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Interstitial lung disease (ILD) is among the most common organ manifestations in collagen vascular diseases and can be a major determinant of the long-term prognosis. Early diagnosis rests mainly on spirometry, including measurement of the CO diffusion capacity, and high-resolution computed tomography (HRCT). ⋯ Therapeutic experience is heterogeneous but evidence is accumulating suggesting that NSIP in the collagen vascular diseases is amenable to immunosuppression. Rapid clinical and/or radiological deterioration, reflecting inflammatory activity, appears to mark those patients who are most likely to benefit from cyclophosphamide therapy.
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Vasculitis and connective tissue disease are often associated with interstitial lung disease. Involvement of lung parenchyma is found in small vessel vasculitis and mostly in ANCA-associated forms such as Wegener's granulomatosis. ⋯ The following article provides an overview of the clinical, histological and radiologic patterns of interstitial lung disease in vasculitis, rheumatoid arthritis and connective tissue disease. Treatment options will also be discussed.