ANCA-negative pauci-immune crescentic glomerulonephritis complicated with recurrent massive gastrointestinal hemorrhage

Abstract

On April 25, 2003, a 62-year-old Japanese man had been admitted to a hospital because of heavy proteinuria and elevated serum creatinine level, and purpura on the lower extremities. On May 15, 2003, he was referred to our hospital for evaluation and treatment. Serum immunoglobulin and complements were within normal ranges. Immune serology was negative for antinuclear antibody, antiglomerular basement membrane antibody, and antineutrophil cytoplasmic antibodies. Histological examination of a percutaneous renal biopsy specimen revealed that all of the glomeruli had severe crescent formation without deposits of immunoreactants. A diagnosis of antineutrophil cytoplasmic antibody-negative pauci-immune crescentic glomerulonephritis was made. The patient was treated with one cycle of steroid pulse therapy (1000 mg methylprednisolone daily, given on 3 consecutive days), and subsequently with prednisolone (60 mg/day). Despite this treatment, renal failure progressed rapidly and hemodialysis was started 1 month after the acute presentation. On May 30, 2003, he suddenly developed massive hematochezia. A technetium-targeted red-blood-cell scan suggested bleeding in the small intestine. On June 11, he presented with massive melena. A bleeding ulcer was found in the third part of the duodenum, and was treated successfully with endoscopy, using a heater probe. On June 19, he presented with massive hematochezia again. Mesenteric angiography revealed active bleeding from the iliac branch of the superior mesenteric artery. He was treated with continuous intraarterial vasopressin infusion by a catheter seated in the branch artery. The majority of patients with pauci-immune crescentic glomerulonephritis, one of the most common causes of rapidly progressive glomerulonephritis, have glomerular disease as part of a systemic vasculitis. Massive gastrointestinal bleeding, although rare, should be considered one of the serious complications in these patients.

AP-VAS 2012 case report: a case of ANCA-negative pauci-immune crescentic glomerulonephritis associated with IL-6-producing adenosquamous cell carcinoma of the lung

“…ANCA also plays a crucial role in the initiation and progression of ANCA-associated CrGN, but a significant number patients with pauci-immune CrGN are negative for ANCA. ANCA-negative pauci-immune CrGN has been demonstrated in only a limited number of case reports [3][4][5][6]. The prevalence of this disease is approximately 10-30 % [3,4].…”

“…Most patients with pauci-immune CrGN have anti-neutrophil cytoplasmic antibodies (ANCAs) in the circulation, so-called ANCAassociated CrGN [2]. However, some patients with this form of glomerulonephritis have no ANCAs [3][4][5][6]. The pathogenesis of ANCA-negative pauci-immune CrGN has not been fully clarified [4].…”

Antineutrophil Cytoplasmic Autoantibody–Negative Pauci-immune Crescentic Glomerulonephritis

“…However, in some patients with pauci-immune CrGN, serum ANCA was negative. Studies on this subgroup of patients were not sufficient as a result of the limited number of patients (7,8,(15)(16)(17).…”

An Unusual Case of Hematuria

“…Limited prevalence data are available on ANCAnegative pauci-immune crescentic glomerulonephritis. A limited number of case series and population-based studies have focused on the clinical spectrum of this disorder (8,(15)(16)(17)(18)(19)(20). In a 10-year retrospective study, the annual incidence rate was 3.9 patients per 1 million individuals and approximately 27% of affected patients with pauciimmune crescentic glomerulonephritis were ANCA negative (18).…”

“…She had periorbital edema as well as bilateral lower extremity pitting edema to the knees. Laboratory evaluation in the emergency room showed normocytic anemia, blood urea nitrogen of 44 mg/dl (normal range [7][8][9][10][11][12][13][14][15][16][17][18], and a creatinine level of 3.8 mg/dl (normal range 0.6 -1.2). Her estimated creatinine clearance was 11.9 ml/minute/1.73 m 2 (normal range 80 -125) with a fractional excretion of sodium of 2.5%.…”