Goodpasture syndrome during the course of a Schönlein-Henoch purpura

doi:10.1053/ajkd.2002.32799

American Journal of Kidney Diseases

Volume 39, Issue 5, May 2002, Page E21

https://doi.org/10.1053/ajkd.2002.32799 Get rights and content

Abstract

Two months after surgical resection of a bronchogenic carcinoma, a 69-year-old patient presented with Schönlein-Henoch purpura with kidney involvement followed by pulmonary hemorrhage. The presence of an IgA linear pattern on the kidney biopsy specimen and circulating anti-glomerular basement membrane (GBM) IgA antibodies led to the diagnosis of Goodpasture syndrome, which implies the possibility that the well-known pulmonary involvement during the course of Schönlein-Henoch purpura could be caused by Goodpasture syndrome in certain cases. In cases of glomerulonephropathy with lung involvement, clinicians should not limit their investigations to anti-GBM IgG. © 2002 by the National Kidney Foundation, Inc.

Section snippets

Case report

A 69-year-old patient had undergone surgery 2 months before admission to our department. The patient had been a smoker up to 2 months before admission, was hypertensive on medical treatment, and had a past medical history of bronchogenic squamous cell carcinoma in the right upper lobe (4.5 cm in diameter, infiltrating parietal pleura, without hilar or mediastinal lymph node involvement). A superior and medial bilobectomy had been performed with mediastinal lymphadenectomy and section of the

Discussion

The diagnosis of Goodpasture syndrome requires the demonstration of anti-GBM circulating antibodies and a characteristic linear pattern on direct immunofluorescence, which is usually IgG, but occasionally non-IgG antibodies can be present or both.⁴ Lung neoplasms have been noted among the precipitating factors that can trigger Schönlein-Henoch purpura.⁵ In the present case, although we did not have pathology samples of the pulmonary lesions, the presence of glomerulonephritis with linear IgA

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The pulmonary-renal syndrome is a rare and life-threatening condition. It is defined as the association of a diffuse alveolar hemorrhage and a rapidly progressive glomerulonephritis. The characteristic histological lesion common to all underlying diseases is a necrotizing and crescentic glomerulonephritis. The pulmonary-renal syndrome is a diagnostic and therapeutic emergency: any delay in its management will lead to death or serious functional damage as pulmonary and renal impairment. ANCA-associated vasculitis and Goodpasture's disease are the main disorders associated to pulmonary-renal syndrome. More rarely systemic lupus, cryoglobulinaemia, Henoch-Schonlein purpura or subacute endocarditis may induce a pulmonary-renal syndrome. Differential diagnosis can sometimes be difficult, highlighting some ambiguity in the definition of the syndrome. Initial treatment usually associates systemic corticosteroid, cyclophosphamide and plasma exchange. The role of biotherapy as first line therapy remains to be determined.
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^☆: Address reprint requests to Luis Carreras, MD, PhD, Scio de Nefrología, Hospital Princeps d'Espanya, CSUB, Feixa Llarga s/n, 08907 L'Hospitalet de Llobregat, Barcelona, Spain. E-mail: [email protected]

^☆☆: 1523-6838/02/3905-0027$35.00/0

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Case ReportGoodpasture syndrome during the course of a Schönlein-Henoch purpura☆,☆☆

Abstract

Section snippets

Case report

Discussion

Am J Med

Am J Kidney Dis

Pulmonary hemorrhage in Henoch Schönlein purpura

Thorax

Henoch-Schönlein purpura and pulmonary hemorrhage: A report and literature review

Pediatr Nephrol

Henoch Schönlein nephritis

Clin Nephrol

IgA-mediated renal diseases: Is there an IgA variant of Goodpasture syndrome?

Am J Kidney Dis

Henoch-Schönlein purpura nephritis

J Am Soc Nephrol

Case Report
Goodpasture syndrome during the course of a Schönlein-Henoch purpura☆,☆☆