Fatal Pulmonary Henöch-Schonlein Syndrome

Kathuria, Satinder; Chejfec, Gregorio

doi:10.1378/chest.82.5.654

Cited by 78 publications

(40 citation statements)

References 5 publications

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“…In one case with pulmonary haemorrhage, leucocytoclastic vasculitis, and probable capillaritis there was extensive granular deposition of IgA along the alveolar septa. 4 We thank Mr J N Fergus for permission to report the case.…”

Section: Discussionmentioning

confidence: 93%

“…Pulmonary capillaritis has not been reported in Henoch-Sch6nlein purpura-although, according to the pathological criteria of Mark and Ramirez, it was present in one of the cases discussed above. 4 The pathological lesion in Henoch-Sch6nlein purpura is a widespread vasculitis, IgA probably having a pathogenetic role. IgA deposits have been found in the mesangium and dermal vessels, and circulating IgA complexes have been found early in the disease.89 Pathological changes in the lung have been observed only in postmortem specimens.…”

Section: Discussionmentioning

confidence: 99%

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Pulmonary haemorrhage in Henoch-Schonlein purpura.

Markus

Clark

1989

Thorax

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A case of Henoch-Schonlein purpura with fatal pulmonary haemorrhage and capillaritis is described.Henoch-Schonlein purpura is a syndrome characterised by a rash, arthritis, gastrointestinal manifestations, and nephritis secondary to diffuse necrotising vasculitis. Clinically evident pulmonary disease is very rare and most reports on series of patients do not mention it. We report a patient with fatal intrapulmonary haemorrhage and briefly review the relevant publications.Case report A 78 year old man was admitted with a one day history of passing dark red blood rectally. Three weeks previously he had been investigated elsewhere for weight loss, arthralgia, and haemoptysis. Chest radiography had shown patchy opacities in both lower lobes. Blood urea and creatinine concentrations were normal. Sputum culture had been negative, and bronchoscopy had showed an inflamed mucosa. He was treated with penicillin on the presumptive diagnosis of bacterial pneumonia and the haemoptysis had resolved.On admission he was shocked, and there was a purpuric rash on the buttocks and lower trunk. The blood urea was 50 mmol/l, potassium 7-4 mol/l, and haemoglobin 16-5 g/dl; the platelet count was normal. Clotting studies showed a prothrombin time of25 (control 10-14) seconds and an activated partial thromboplastin time of 52 (control 30-45) seconds. A chest radiograph was normal.The patient's condition improved rapidly after resuscitation with intravenous fluids, and the potassium concentration fell to normal values, though the urea remained raised. Oesophagogastroscopy showed haemorrhagic ulcerated oesophagitis. Vitamin K was given and clotting studies gave normal results by the next day.He remained clinically stable until the third day after admission, when, over minutes, he became acutely dyspnoeic. Chest examination revealed right basal crepitations. Antibiotics were started, but over the next hour he became increasingly breathless and died.At necropsy the lungs were heavy, oedematous, and congested. Microscopically there was extensive intraAddress for reprint requests:

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Section: Discussionmentioning

confidence: 93%

Section: Discussionmentioning

confidence: 99%

Pulmonary haemorrhage in Henoch-Schonlein purpura.

Markus

Clark

1989

Thorax

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“…The principal cause of pulmonary hemorrhage in HSP is vasculitis of small pulmonary blood vessels, especially capillaries [9][10][11] . Corticosteroids have been used with the hope of ameliorating the vasculitis, but efficacy has not been established.…”

Section: Discussionmentioning

confidence: 99%

A Case of Pulmonary Hemorrhage Associated with Henoch-Schönlein Purpura

Sim

Choi

et al. 2009

Tuberc Respir Dis

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Henoch-Schönlein purpura (HSP) is an immunologically mediated systemic vasculitis of small blood vessels that primarily involves the skin, gastrointestinal tract, joints and kidneys. HSP is a common vasculitic syndrome in children who, in most cases, achieve complete recovery. Pulmonary hemorrhage is a very rare manifestation of HSP. The authors present a case of a 46-year-old male presenting with pulmonary hemorrhage and renal involvement and the diagnosis of HSP. The patient responded to prednisolone therapy.

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“…Pulmonary-renal syndrome in an adult due to HSP confirmed by histology and immunohistochemistry has only been reported in four patients in the literature, two of whom died and two of whom responded to treatment with prednisolone [6][7][8][9]. In addition, two papers have described patients with suspected HSP and pulmonary haemorrhage, but the absence of immunoglobulins and C3 in the mesangium at necropsy cast doubt on the diagnosis in the first case and, in the second, the authors could not demonstrate vasculitis in the lungs [10][11][12].…”

Section: Discussionmentioning

confidence: 99%

A 47-year-old male with respiratory and renal failure

Jaiswal

Packham

Stevens

et al. 2005

Breathe

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A 47-year-old male was admitted with a 5-day history of painful, red, swollen legs, and 48 hours of intermittent sweats and rigors. His only previous medical history was Perthe's disease of the right hip at the age of 8 years. He had no allergies and there was no family history. He was taking ampicillin at a dose of 500 mg t.d.s., as prescribed by his general practitioner. He had a temperature of 39°C on admission. Physical examination proved normal, apart from the area of skin over both ankles and the lower right leg, which was swollen, red, hot and tender.Initial investigations revealed leukocytosis of 15.9x10 -9 , neutrophils 11.7x10 -9 and a C-reactive protein level of 70.5 (normal range 0-10). Renal function and serum electrolytes were normal. Chest and ankle radiographs were also normal. Hip radiography revealed changes consistent with old Perthe's disease. Blood cultures were negative. A diagnosis of cellulitis was made by the admitting physicians, and the patient was prescribed intravenous benzylpenicillin and flucloxacillin.The leukocytosis resolved, but the patient continued to experience a fluctuating temperature. Nine days after admission, he developed non-specific abdominal pains. An ultrasound of the abdomen, kidneys and renal tract proved normal. The next day, he developed a rash on both legs (figure 1).

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Fatal Pulmonary Henöch-Schonlein Syndrome

Cited by 78 publications

References 5 publications

Pulmonary haemorrhage in Henoch-Schonlein purpura.

Pulmonary haemorrhage in Henoch-Schonlein purpura.

A Case of Pulmonary Hemorrhage Associated with Henoch-Schönlein Purpura

A 47-year-old male with respiratory and renal failure

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