Algorithm for diagnosis of primary vasculitides

Abstract

Primary vasculitides are diseases with a wide variety of anatomical, clinical, radiological, and laboratory presentations. Primary vasculitides are difficult to diagnose because of the complexity of clinical presentation, which may lead to delayed treatment and increased financial costs of workup investigations involving non-essential tests. Our objective in the present study is to create an algorithm that helps diagnosis of Primary vasculitides. The algorithm presented in this article allows fast, simple and cost-effective diagnosis of primary vasculitides using just clinical concepts and a few laboratory tests.

Diagnóstico e terapêutica precoce de vasculite leucocitoclástica: relato de caso

“…In addition to their low specificity and high cost, these tests can also delay definitive diagnosis. 7,16 The American College of Rheumatology consensus bases diagnosis of CLV on the following criteria: -Histopathological study encompassing arterioles and venules with presence of perivascular or extravascular granulocytes.…”

“…1,17 Use of the algorithm mentioned above was of fundamental importance to making a rapid and economically feasible diagnosis. 7 Cutaneous biopsy may be indispensable to diagnosis of this disease. In addition to assessing the caliber of the vessels involved, pathology will also evaluate the possibility of other diagnostic hypotheses such as perivasculitis, embolic phenomena, and other non-inflammatory vasculopathies that simulate vasculitis.…”

“…The Bezerra algorithm was consulted (illustrated in Figure 2), observing that ANCA P, ANCA C, cryoglobulins, and IgA were all negative, suggesting a diagnosis of CLV. 7 After definition of this diagnosis, on 29 September, 2017, amputations were performed of both halluxes and the distal phalanx of the 2nd, 3rd, and 4th toes of the right foot, ordering pathology of the tissues amputated from the feet, which confirmed the diagnosis of CLV, by "fragmentation of neutrophil nuclei". Treatment was initiated with methylprednisolone, 20 mg every 6 h for 3 days, cilostazol 100 mg every 12 h, and cyclophosphamide monthly, in a single 750 mg dose.…”

“…Use of a systematic workup process is very important to hasten the start of treatment, improving prognosis. 1,7 Diagnosis is confirmed by histopathology, with findings of inflammatory infiltrate and fragmentation of the nuclei of neutrophils in the vascular walls, which is known as karyorrhexis and is related to fibrinoid necrosis. 3,8 This report describes a case of CLV in which a systematic algorithm was used for diagnostic investigation, enabling early treatment with good results.…”

“…3,8 This report describes a case of CLV in which a systematic algorithm was used for diagnostic investigation, enabling early treatment with good results. 7…”