Large vessel involvement in ANCA-associated vasculitides: report of a case and review of the literature

Abstract

Vasculitides are currently classified according to the size of the vessels involved and characteristic clinical and histopathologic findings. Antineutrophil cytoplasmic antibodies (ANCA) and other serologic tests have been used to further characterize small vessel vasculitides. Large vessel involvement in ANCA-associated small vessel vasculitides has been overlooked in the medical literature. Here, we report a case of fatal aortitis and aortic dissection in a patient with microscopic polyangiitis and review reported cases of large vessel involvement in ANCA-associated vasculitides since 1990. We have attempted to characterize this subgroup of patients. Large vessel disease in ANCA-associated vasculitis may present as stenosing large vessel arteritis, aneurysmal disease, aortic dissection, aortic rupture, aortic regurgitation, and death. Prominent perivascular inflammation may present as mediastinal, cervical or abdominal soft tissue masses. ANCA-associated large vessel disease should be considered in the differential diagnosis of these disorders. The epidemiologic, clinical and pathologic characteristics of these patients differ from those of the well-defined large vessel vasculitides such as giant cell (temporal) arteritis or Takayasu's arteritis. We suggest that large vessel involvement is part of the spectrum of ANCA-associated vasculitis rather than an overlap with other large vessel vasculitides. It occurs in both myeloperoxidase- and proteinase 3-positive patients with either Wegener's granulomatosis or microscopic polyangiitis, but has not been reported in Churg-Strauss syndrome. Large vessel vasculitis can precede small vessel vasculitis or occur in the absence of small vessel involvement. We hope this report will contribute to the ongoing development of classification systems for the vasculitic syndromes.

Concurrent Takayasu’s Arteritis and Antineutrophil Cytoplasmic Antibody-related Glomerulonephritis Related to Use of Propylthiouracil: Figure 1.

“…Further, there have appeared cases of concurrent large-vessel and ANCA-associated small-vessel vasculitis, although these have not been previously linked to PTU use [10][11][12][13] . It has been noted in these cases that when ANCA-associated small-vessel vasculitis and aortitis coexist, the aortitis is characterized more by features of aneurysm formation and dissection than by stenotic lesions 14 , as occurred in this case.…”

Aortitis, causas infrecuentes en Reumatología: presentación de casos

“…Dados estos antecedentes, los síntomas con los que debutó fueron atribuidos a la aortitis y se concluyó que esta era secundaria a la misma enfermedad, ya que no existía otra causa que la explicara. Si bien las vasculitis asociadas a ANCA afectan predominantemente vasos de pequeño calibre, existen reportes de compromiso aórtico y otros grandes vasos [12][13][14][15][16] . En aquellos con estudio histopatológico se encontró infiltrado inflamatorio mixto, especialmente alrededor de vasa vasorum y sólo algunos tenían presencia de granulomas 16 .…”

Vasculitis de grandes vasos en paciente con vasculitis ANCA (+): Caso clínico

“…Se prescribió antibióticos y se citó a control en 1 semana. Reapareció 6 meses más tarde con notorio compromiso del estado general, falla renal (creatinina: 7,4 mg/dl, clearance de creatinina:12 ml/min/1,73m 2 ; uremia: 185 mg/dl) y proteinuria: 4.534 mg/24 h). La serología para virus B, C y VIH fue negativa.…”

“…Las vasculitis de vasos grandes, en cambio, no los tienen 1,2 . El compromiso de vasos grandes en las vasculitis de vasos pequeños ANCA(+) es raro [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18] . Algunos autores consideran que es una coincidencia de dos patologías, otros creen que no lo es, sino que existe una patogénesis común 1,4,12 .…”