Henoch-Schönlein purpura with rapidly progressive glomerulonephritis and fatal intraperitoneal hemorrhage in an adult.
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Abstract:
We report here the autopsy findings in a 51-year-old man who had been admitted with Henoch-Schönlein purpura (HSP) accompanied by rapidly progressive glomerulonephritis and massive intraperitoneal hemorrhage, leading to death. While the intraperitoneal hemorrhage was the primary cause of death, the patient may have suffered widespread intraperitoneal vasculitis due to HSP, or hemorrhagic pancreatitis due to the concurrent administration of a steroid and furosemide. We emphasize the acute hemorrhagic pancreatitis is a possible complication in patients with generalized vasculitis, including HSP and collagen disease, during the concurrent administration of steroids and other agents.Keywords:
Purpura (gastropod)
Rapidly progressive glomerulonephritis
Polyarteritis Nodosa
Intraperitoneal injection
Collagen disease
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We report here the autopsy findings in a 51-year-old man who had been admitted with Henoch-Schönlein purpura (HSP) accompanied by rapidly progressive glomerulonephritis and massive intraperitoneal hemorrhage, leading to death. While the intraperitoneal hemorrhage was the primary cause of death, the patient may have suffered widespread intraperitoneal vasculitis due to HSP, or hemorrhagic pancreatitis due to the concurrent administration of a steroid and furosemide. We emphasize the acute hemorrhagic pancreatitis is a possible complication in patients with generalized vasculitis, including HSP and collagen disease, during the concurrent administration of steroids and other agents.
Purpura (gastropod)
Rapidly progressive glomerulonephritis
Polyarteritis Nodosa
Intraperitoneal injection
Collagen disease
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Citations (8)
Polyarteritis Nodosa
Microscopic polyangiitis
Purpura (gastropod)
Granulomatosis with polyangiitis
Skin biopsy
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Citations (2)
Microscopic polyangiitis (MPA) can be distinguished from Henoch‐Schoenlein purpura (HSP) based on the presence of renal‐pulmonary complications, myeloperoxidase‐antineutrophil cytoplasmic antibody (MPO‐ANCA) seropositivity and pauci‐immune glomerulonephritis; these characteristics of MPA are generally not found in HSP. Here, we present a unique case with MPA and HSP overlapping syndrome. A 74‐year‐old man presented with a skin rash accompanied by intracranial bleeding, acute renal failure and pulmonary hemorrhage resulting in a fatal outcome. An autopsy revealed the marked formation of crescentic glomerulonephritis, diffuse pulmonary alveolar hemorrhage and focal cerebral bleeding. Histological features showing both dermal small‐vessel vasculitis and cutaneous polyarteritis nodosa‐like arteritis suggested MPA rather than HSP, in which the dermal small vessels are exclusively affected. Meanwhile, capillary immunoglobulin (Ig)A deposits in the skin and glomeruli suggested HSP. As MPA and HSP overlapping syndrome characterized by the synergistic effect of MPO‐ANCA and the IgA immune complex may result in a fatal outcome, aggressive immunosuppressive therapy should be initiated as early as possible.
Microscopic polyangiitis
Purpura (gastropod)
Pulmonary hemorrhage
Fibrinoid necrosis
Systemic vasculitis
Polyarteritis Nodosa
Rapidly progressive glomerulonephritis
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Purpura (gastropod)
Acute glomerulonephritis
Rapidly progressive glomerulonephritis
Presentation (obstetrics)
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Polyarteritis Nodosa
Purpura (gastropod)
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Henoch‐Schönlein purpura (HSP) is a rather common disease characterized by systemic hypersensitivity vasculitis in the skin and other visceral organs. It has a favorable prognosis unless it is complicated by severe glomerular disease. We report a distinctive fatal case of systemic vasculitis combined with HSP and polyarteritis nodosa (PN) in a 56‐year‐old man who died of progressive renal failure one month after the onset of the disease. He complained of arthralgia, purpura of both lower extremities, nasal bleeding and tarry stool, and acute renal failure was noted at the time of admission to hospital. A skin biopsy from the purpura lesion exhibited leucocytoclastic vasculitis with IgA deposition, and HSP was considered. However, renal failure progressed rapidly and subsequently was complicated by acute myocardial infarction. Postmortem examination revealed PN type necrotizing vasculitis in the kidneys, heart and mesentery resulting in acute multiple infarctions of these organs. We think the current case was a polyangitis overlap syndrome. It is important to suspect the polyangitis overlap syndrome positively when progressive acute renal failure is seen in a patient with HSP and to begin appropriate therapy immediately.
Polyarteritis Nodosa
Purpura (gastropod)
Palpable purpura
Necrotizing Vasculitis
Systemic vasculitis
Rapidly progressive glomerulonephritis
Skin biopsy
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Citations (27)
Henoch–Schönlein purpura glomerulonephritis (HSP-GN) is a common form of systemic small vessel vasculitis in children. Although prognosis is usually favourable, the disease is occasionally associated with a risk of renal insufficiency. Various immunosuppressive agents have been used in patients with severe HSP-GN, but none have shown convincing favourable effects. We report a case of biopsy-proven HSP-related GN in a 4-year-old girl that responded remarkably well to cyclosporine A (CsA), following failure to respond to other immunosuppressive agents. At 8 months post-CsA treatment, repeat renal biopsy findings were consistent with histological improvement. We conclude that CsA treatment not only exerts beneficial effects on resistant HSP-related GN but may also arrest progression of the disease.
Nephritis
Purpura (gastropod)
Rapidly progressive glomerulonephritis
Skin biopsy
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Citations (2)
A 77-year-old male diagnosed as having Henoch-Schönlein purpura (HSP) died of acute respiratory and renal failure. Autopsy revealed necrotizing glomerulonephritis with IgA deposition in mesangium and capillary walls of glomeruli. The lungs showed leukocytoclastic vasculitis of the alveolar walls and immunohistochemically revealed IgA deposition and ultrastructurally electron-dense deposits in the capillary basement membrane and interstitium of the alveoli. Moreover, fibrinoid necrosis of small and medium-sized arteries were observed in the kidneys, bronchial arteries of the lungs, stomach and small intestine. We have concluded that this case may be diagnosed as polyangitis overlap syndrome in an elderly man consisting of HSP and polyarteritis nodosa (PN), and the pulmonary hemorrhage may be significantly relevant to the pulmonary lesions not in PN but in HSP.
Polyarteritis Nodosa
Purpura (gastropod)
Fibrinoid necrosis
Mesangium
Pulmonary hemorrhage
Necrotizing Vasculitis
Palpable purpura
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Citations (21)
A 76 year old white woman died from massive pulmonary haemorrhage nine days after she was admitted to Parkland Memorial Hospital for evaluation of haemoptysis and rapidly progressive glomerulonephritis. The differential diagnosis of haemoptysis with rapidly progressive glomerulonephritis is presented with particular emphasis on Wegener9s granulomatosis and microscopic polyarteritis. Necropsy showed a small vessel necrotising vasculitis associated with a focal segmental necrotising glomerulonephritis consistent with microscopic polyarteritis.
Rapidly progressive glomerulonephritis
Polyarteritis Nodosa
Etiology
Purpura (gastropod)
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Citations (20)
Polyarteritis Nodosa
Proteinase 3
Nephritis
Microscopic polyangiitis
Rapidly progressive glomerulonephritis
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Citations (2)