Pulmonary manifestation in churg-strauss syndrome: clinical case

Abstract

Systemic connective tissue disorders still remain one of the great diagnostic challenges in real practice because they often begin with nonspecific symptoms and signs and progresses slowly over months and even years. Vasculitisrefers to a heterogeneous group of disorders that is characterized by inflammatory lesion of blood vessels. Depending on size, distribution, and severity of the affected vessels, vasculitis can result in clinical syndromes that vary in severity from a minor self-limited rush to a life-threatening multisystem disorder. Current clinical classification of primary vasculitides is based on clinicopathologic features: they can be separated by the size of affected vessels (large, medium, small).1 Small-vessel vasculitides are associated with antineutrophil cytoplasmic antibodies (ANCA), which can be detected in plasma.2,3 One of the common clinical types of ANCA-associated vasculitis is Churg-Strauss syndrome (CSS) reported at first time by Churg and Strauss in 1951. Asthma, eosinophilia, and systemic vasculitis are the hallmarks of the ChurgStrauss syndrome. The Chapel Hill Consensus Conference defined CSS as a disorder characterized by eosinophil-rich, granulomatous inflammation of the respiratory tract and necrotizing vascilitis of smallto medium vessels with asthma and eosinophilia.1,4 Since lung involvement is a frequent clinical feature of ANCA-associated vasculitides the differential diagnosis should be made to verify systemic vasculitis in patients with pulmonary disorder.3,5 General clinical clues suggesting the presence of systemic vasculitis are constitutional symptoms, signs of inflammation, subacute onset, joint pain, multisystem disease evident. Establishing the diagnosis of vasculitis requires confirmation by laboratory test, biopsy, and/or serology. In term to demonstrate the typical natural history of ANCAassociated vasculitis and diagnostic difficulties in real clinical practice we present observed clinical case.