Hemorrhagic complications associated with PR3-ANCA crescentic glomerulonephritis

Figures & data

Figure 1. Chest X-ray (A) and CT (B) revealed large areas of ground glass opacity in the middle and lower lung fields. Abdominal CT (C and D) showed an extensive, irregular low-density mass in the right rectus muscle (white arrow). Gastrointestinal (GI) endoscopy demonstrated two duodenal ulcers around 2 cm diameter. Some of the mucosa between the ulcers is reddish and edematous. The border of the ulcer is unclear and covered with white coating (E). Another ulcer is square and clears (F). Renal biopsy showed two large fibrocellular crescent (black arrow) and a small cellular crescent (black thin arrow) in the renal glomeruli (G and H). Protein cast were presented with lymphomonocyte interstitial infiltration around glomeruli (H). Periodic acid-Schiff stain, 200×.

Figure 2. Clinical course of the patient. Scr, serum creatinine; Hb, hemoglobin; UGIH, upper gastrointestinal hemorrhage; PE, plasma exchange; MP, methylprednisolone.

Table 1. The clinical manifestations of intra-cerebral hemorrhage in patients with ANCA disease.

No.	Sex/Age	ANCA pattern	Diagnosis	CNS involvement	Treatment	Outcome	Reference
1	F/10	MPO-ANCA	MPA	Hemorrhagic focus in the right parietal lobe	Seven cycles of plasmapheresis, steroid and cyclophosphamide therapy	The neurological settled but dialysis-dependent	Tan23
2	F/62	MPO-ANCA	MPA	Hemorrhage in the right frontal lobe, a second Hemorrhage was also noticed in the left frontal lobe	Steroid and cyclophosphamide therapy	Death	Akkara Veetil24
3	M/48	MPO-ANCA	MPA	Massive right-sided parieto-temporo-occipital Hematoma with cerebral hernia and intraventricular hemorrhage	Surgical evacuation of the hematoma, steroid and cyclophosphamide therapy	Full recovery other than a persistent bilateral foot drop	Sassi25
4	M/43	MPO-ANCA	MPA	Massive intra-cerebral hemorrhage	Methylprednisolone and cyclophosphamide	Death	Han26
5	M/56	p-ANCA	MPA	Massive cerebral hemorrhage	Steroid therapy alone	Death	Ito27
6	F/44	MPO-ANCA	MPA	Subarachnoid hemorrhage, a saccular-like aneurysm from the right distal posterior inferior cerebellar artery	Two operations, steroid, and cyclophosphamide therapy	Full recovery (modified Rankin scale score 1)	Kimura28
7	M/46	PR3-ANCA	GPA	Right thalamic hemorrhage	Steroid, and cyclophosphamide therapy	Recovery	Takaoka22

Notes: ANCA, anti-neutrophil cytoplasmic autoantibody; CNS, central nervous system; GPA, granulomatosis with polyangiitis; MPA, microscopic polyangiitis; MPO, myeloperoxidase; PR3, proteinase 3.

Table 2. The clinical manifestations of gastrointestinal hemorrhage in patients with ANCA disease.

No.	Sex/Age	ANCA pattern	Diagnosis	GI involvement	Histology	Outcome	Reference
1	M/46	NK	GPA	Small bowel, colon	Ulceration	Death	Geraghty33
2	M/50	NK	GPA	Distal ileum	Ulceration	Good (after surgery, follow 6 mo)	McNabb34
3	M/24	cANCA	GPA	Colon	Vasculitis	Good (after surgery, follow 3 mo)	Storesund35
4	F/55	cANCA	GPA	Small bowel, colon	Vasculitis	Good (after surgery, follow 2 mo)	Storesund35
5	M/44	NK	GPA	Small bowel	Vasculitis	Good (after surgery, follow 4 mo)	Deniz36
6	M/57	MPO-ANCA	MPA	Duodenal	Vasculitis	Death	Wakura37
7	M/54	MPO-ANCA	MPA	Ileum	Vasculitis	Good	Inaguma38
8	M/19	NK	EGPA	Appendix	Vasculitis	GI relapse (after surgery 24 mo), then good (120 mo)	Pagnoux40
9	F/39	NK	MPA	Small intestine, large bowel	Vasculitis	Multiple relapses, and cerebral vasculitis (after surgery 132 mo), ongoing regression (140 mo)	Pagnoux40
10	F/43	NK	MPA	Small intestine, appendix	Vasculitis	Good (after surgery, follow 6 mo)	Pagnoux40

Notes: ANCA, anti-neutrophil cytoplasmic autoantibody; GPA, granulomatosis with polyangiitis; MPA, microscopic polyangiitis; EGPA, eosinophilia granulomatosis with polyangiitis; MPO, myeloperoxidase; M, male; F, female; NK, not known.

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