Jörg Kaufmann1, Gert Hein, Günter Stein. 1. Klinik für Innere Medizin IV, Funktionbereich Rheumatologie und Osteologie, Friedrich-Schiller-Universität Jena. joerg.kaufmann@med.uni-jena.de
Abstract
BACKGROUND: Hypersensitivity vasculitis (leukocytoclastic vasculitis) is defined as small-vessel vasculitis mediated by deposition of immune complexes (Arthus reaction) after exposition to various agents, such as drugs, toxins and infections. Due to a wide spectrum of precipitating agents and symptoms, classification systems and synonyms, understanding of the disease, its diagnosis and therapeutic strategy are difficult. METHODS: Based on an extensive analysis of the literature, causal agents, etiopathogenesis as well as symptoms and their frequencies are summarized. The widespread differential diagnoses and the development of a diagnostic strategy for practical management of the disease are discussed. RESULTS AND CONCLUSIONS: The course of disease is generally benign, and spontaneous remission is often observed. Severe organ manifestations and chronic courses have, however, been described. Clinical diagnosis must be confirmed histopathologically. Once the diagnosis is made, search for etiologic agents (successful in only 50% of the cases) and an intensive organ diagnostics should be performed. Subsequently, treatment with steroids, antihistamines and slow-acting antirheumatic drugs such as cytotoxic substances and immunosuppressive drugs is planned according to severity and prognosis.
BACKGROUND:Hypersensitivity vasculitis (leukocytoclastic vasculitis) is defined as small-vessel vasculitis mediated by deposition of immune complexes (Arthus reaction) after exposition to various agents, such as drugs, toxins and infections. Due to a wide spectrum of precipitating agents and symptoms, classification systems and synonyms, understanding of the disease, its diagnosis and therapeutic strategy are difficult. METHODS: Based on an extensive analysis of the literature, causal agents, etiopathogenesis as well as symptoms and their frequencies are summarized. The widespread differential diagnoses and the development of a diagnostic strategy for practical management of the disease are discussed. RESULTS AND CONCLUSIONS: The course of disease is generally benign, and spontaneous remission is often observed. Severe organ manifestations and chronic courses have, however, been described. Clinical diagnosis must be confirmed histopathologically. Once the diagnosis is made, search for etiologic agents (successful in only 50% of the cases) and an intensive organ diagnostics should be performed. Subsequently, treatment with steroids, antihistamines and slow-acting antirheumatic drugs such as cytotoxic substances and immunosuppressive drugs is planned according to severity and prognosis.