Development of intracerebral hemorrhage in the short-term clinical course of a patient with microscopic polyangiitis without neurological symptoms at diagnosis: an autopsy case

Abstract

A 77-year-old man with high-grade fever, progressive renal dysfunction, high serum level of C-reactive protein and positive serum myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA) was diagnosed with microscopic polyangiitis with rapidly progressive glomerulonephritis, and remission induction treatment with glucocorticoids and intravenous cyclophosphamide was initiated. Although his general condition improved in a short time, intracerebral hemorrhage occurred 12 days after the initiation of treatment and emergent hematoma evacuation was performed. However, he passed away on day 14. Surprisingly, even though no clinical findings for any organs except for renal involvement was detected before his death, autopsy revealed necrotizing vasculitis affecting various systemic organs including kidney, pancreas, liver, myocardium in ventricle, adipose tissue of the left adrenal gland, small intestine, gallbladder, bronchus, prostate, testis and spleen. It is difficult to detect widespread vasculitis without clinical symptoms and signs in patients with ANCA-associated vasculitis. A whole body assessment tool is necessary to detect unexpected vital organ damage, including cerebral vessels.

Keywords: Autopsy; Intracerebral hemorrhage; Microscopic polyangiitis; Myeloperoxidase-antineutrophil cytoplasmic antibody.

Fig. 1

Clinical course. CFPM cefepime; PSL prednisolone; IVCY intravenous pulse cyclophosphamide

Fig. 2

Cranial CT imaging showing an acute lesion of left-sided intracerebral hemorrhage that ruptured into the lateral ventricle

Fig. 3

a Autopsy revealed inflammatory involvement with fibrinoid necrosis of a small vessel in the brain. (Hematoxylin and eosin stain, original magnification ×200). b Crescent formation is seen in approximately 2 % of glomeruli. Angiitis of the small vessels with fibrinoid necrosis was observed, but there is no marked glomerular change in this region (hematoxylin and eosin stain, original magnification ×100)

Fig. 4

Autopsy revealed necrotizing vasculitis affected in many organs including (a) pancreas, (b) liver, (c) ventricle in his heart, (d) adipose tissue of the left adrenal gland, (e) small intestine, (f) gallbladder, (g) bronchus, (h) prostate, (i) testis, and (j) spleen