Pseudovasculitis: Mechanisms of vascular injury and clinical spectrum

Abstract

Pseudovasculitis, vasculitis-like syndromes, or mimics of vasculitis represent a heterogeneous collection of disorders that are capable of simulating a vasculitic disorder. Some conditions such as cardiac myxomas, cholesterol embolization, and fibromuscular dysplasia are more apt to cause confusion, but numerous other conditions can do so also. Inappropriate diagnosis leads to delay or absence of proper management and exposure to potentially deleterious treatment modalities such as corticosteroids and cytotoxic agents. The diagnosis of a pseudovasculitic disorder requires a high index of suspicion and should always be part of the differential diagnosis of vasculitis. The endothelium is thought to be pivotal in vascular injury; much has been learned using in vitro human umbilical vein endothelial cell cultures. Application of this knowledge to human disease and to vasculitic disorders and their imitators is still premature.

Vascular purpura revealing a severe dilated cardiomyopathy with left ventricular apical thrombus

“…Diagnostic overlap between vasculitis and heterogenous disorders capable of simulating similar skin lesions[2] might explain that systemic embolisms as well as in situ thrombotic disorders can be revealed by rapidly extensive purpura mimicking cutaneous vasculitides. [34]…”

Displasia fibromuscular: um diagnóstico diferencial para as vasculites

“…10 In addition, involvement of the following arteries has been reported: visceral; iliac; subclavian; brachial; and popliteal. 1,2,4,11,12 …”

“…The involvement of other arteries can remain asymptomatic. 2,10,11 In patients with FMD of renal artery, because of arterial obstruction, ischemia and progressive loss of the renal parenchyma occur, in addition to the clinical fi ndings of arterial hypertension. 13 The neurological manifestations comprise transient ischemic attacks, cerebral vascular accident, subarachnoid hemorrhage, and unspecifi c fi ndings, such as headache, vertigo, tinnitus, hemianopsia, 9 ataxic paraparesis, 14 and mental alterations, depending on the brain area affected.…”

“…[16][17][18] The diagnosis of FMD is established by histopathology or angiography. 2,11 The latter, in addition to diagnosing, suggests the arterial layer affected, because each arterial layer has a differentiated pattern on the imaging study. Involvement of the media is seen as the classical pattern of "pearl necklace", in which sequential thickening and thinning of the affected arterial segment occurs.…”

Vasculitis-like syndromes

“…Cardiac myxomas and cholesterol emboli caused by endothelial-induced vascular injury have also been reported to mimic cutaneus vasculitis [26].…”