Pseudovasculitis and corticosteroid therapy

Abstract

Pseudovasculitis, vasculitis-like syndromes, vasculitis look-alikes, or mimics of vasculitis represent a heterogeneous collection of disorders that are capable of simulating vasculitis. Inappropriate diagnosis leads to delay or absence of proper management and exposure to potentially deleterious treatment modalities such as corticosteroids and cytotoxic agents. We report the case of fibromuscular dysplasia suspected to be a polyarteritis nodosa. The progression of the lesions visualized by the ultrasonographic study and computed tomography (CT) scan after 10 days of treatment led to an emergency laparotomy. The possible deleterious role of steroids given to treat the suspected vasculitis is discussed.

Displasia fibromuscular: um diagnóstico diferencial para as vasculites

“…Angiography and biopsy should be performed whenever possible. 10,[22][23][24] Histological examination is often considered gold-standard. One characteristic histological image can confi rm a diagnosis of vasculitis and exclude other diseases, such as neoplasia and infection.…”

“…In such situations, the differential diagnosis should always include pseudovasculitis. [22][23][24] There are isolate reports of FMD mimicking PAN, Ehlers-Danlos's syndrome, Alport's syndrome, pheochromocytoma, Marfan's syndrome, and Takayasu's arteritis (TA).…”

“…2 Less frequently, cerebrovascular accident and skin involvement (palpable purpura, livedo reticularis, necrotic lesions and infarctions of the fi nger tips) can occur. 22 The classifi cation criteria comprise clinical and non-clinical elements. Of the later, the discovery of several aneurysmatic dilations up to 1 cm on visceral angiography is considered suffi cient for the diagnosis of PAN, even in the absence of histological evidence of the disease, which leads to confusion with FMD.…”

“…2 Laboratory tests, such as antinuclear antibodies, rheumatoid factor, and ANCA, are usually negative in PAN, which makes the differential diagnosis even more diffi cult. 22 TA and FMD are vaso-occlusive diseases, and the signs and symptoms of both conditions refl ect some degree of damage to the extremity of the organ. The presence of infl ammation should suggest the diagnosis of TA; however, in the chronic vaso-occlusive phase of the disease, most patients have no infl ammatory signs.…”