PubMed · 9047980
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O Aizawa, S Mochizuki, M Kawakami. 1996. [Systemic necrotizing angitis, hypersensitivity vasculitis].. https://pubmed.ncbi.nlm.nih.gov/9047980/
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Polyarteritis nodosa (PAN), first described by Küssmaul and Maier, is a well-known form of necrotizing angiitis whose manifestations are weight loss, fever, asthenia, peripheral neuropathy, renal involvement, musculoskeletal and cutaneous manifestations, hypertension, gastrointestinal tract involvement, and cardiac failure. Recently individualized from PAN, microscopic polyangiitis (MPA) is a systemic vasculitis of small-size vessels whose clinical manifestations are very similar to those of PAN, but it is characterized by the presence of rapidly progressive glomerulonephritis (RPGN), which is nearly constant, and pulmonary involvement usually absent in PAN. Churg Strauss syndrome (CSS) is a disorder characterized by hypereosinophilia and systemic vasculitis similar to that of PAN and occurring in individuals with asthma and allergic rhinitis. Considering the etiologies of PAN, primary and secondary vasculitides can also be distinguished because PAN can be the consequence of hepatitis B virus (HBV) infection and sometimes of other etiologic agents. The prognosis of systemic vasculitides has been transformed by corticosteroids that are, except in HBV-related PAN, the basic treatment. Immunosuppressive drugs, especially cyclophophamide, have also contributed to improving the prognosis, but their precise role in the management of these vasculitides is still being elucidated.
OBJECTIVE: The aim of this study was to evaluate the pulmonary CT findings in patients with Churg-Strauss syndrome to determine the frequency and nature of parenchymal abnormalities. MATERIALS AND METHODS: CT examinations performed at the time of diagnosis in 17 patients with Churg-Strauss syndrome were retrospectively evaluated by two observers who reached a decision by consensus about the presence and nature of parenchymal abnormalities. High-resolution CT (1- to 3-mm collimation) was performed in 14 patients and conventional CT (6- to 10-mm collimation) was performed in three cases. RESULTS: Predominant CT findings consisted of parenchymal opacification (consolidation or ground-glass attenuation) (n = 10), pulmonary nodules (n = 2), bronchial wall thickening or dilatation (n = 2), interlobular septal thickening (n = 1), and normal anatomy (n = 2). Parenchymal opacification was predominantly peripheral (n = 6) or random in distribution (n = 4). CONCLUSION: The most common CT finding in patients with Churg-Strauss syndrome consists of areas of parenchymal opacification that may be random or peripheral in distribution. These findings are nonspecific.
With the opportunity for long-term follow-up, it has been appreciated that disease severity may vary considerably in patients with Wegener's granulomatosis, polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome. Although combined therapy with cyclophosphamide and glucocorticoids continues to be the foundation of treatment, concerns have increased about the toxicities of this regimen. As illustrated by studies on Wegener's-related subglottic stenosis and endobronchial involvement, it has also become apparent that some disease manifestations may not respond to this therapy. Recent therapeutic investigations have therefore focused on identifying approaches that are less toxic and that are based on anatomic involvement and disease severity. The study of polyarteritis nodosa has additionally been affected by proposed changes in classification that would make this condition a separate entity from microscopic polyangiitis. As progress is made in defining the clinical significance of this nomenclature, the prognostic factors in these patients have also been examined, which may be helpful in guiding therapeutic decisions.