Pulmonary Hemorrhage and Crescentic Glomerulonephritis in a Patient with Seropositive Anti-Glomerular Basement Membrane Disease and Anti-Neutrophil Cytoplasmic Antibodies
Anti-glomerular basement membrane (anti-GBM) disease is an acute and life-threatening systemic autoimmune disorder. The coexistence of circulating anti-neutrophil cytoplasmic antibodies (ANCA) and anti-GBM disease, the so-called double-positive disease (DPD), is exceptionally rare. We report a un...
| Main Authors: | , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Sestre Milosrdnice University hospital, Institute of Clinical Medical Research
2022-01-01
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| Series: | Acta Clinica Croatica |
| Subjects: | |
| Online Access: | https://hrcak.srce.hr/file/428589 |
| Summary: | Anti-glomerular basement membrane (anti-GBM) disease is an acute and
life-threatening systemic autoimmune disorder. The coexistence of circulating anti-neutrophil cytoplasmic
antibodies (ANCA) and anti-GBM disease, the so-called double-positive disease (DPD), is
exceptionally rare. We report a unique case of DPD manifesting as pulmonary-renal syndrome (PRS)
in a 46-year-old woman who first presented with clinical and radiological suspicion of pneumonia.
Chest computed tomography scan later revealed bilateral alveolar hemorrhage. Kidney biopsy showed
necrotizing crescentic (100% glomeruli) glomerulonephritis. On immunofluorescence microscopy,
glomeruli were global linear positive for IgG, confirming anti-GBM disease. Double positivity was
detected for circulating anti-myeloperoxidase ANCA (p-ANCA) and anti-GBM antibodies. Acute
renal failure evolved rapidly. Therapeutic plasma exchange (TPE) and hemodialysis (HD) were initiated
early in combination with intravenous pulse corticosteroid therapy followed by oral methylprednisolone
and cyclophosphamide. Pulmonary hemorrhage resolved, but renal function could not be
preserved. The patient remains HD dependent. This case report highlights that pulmonary symptomatology
may be the leading clinical presentation of PRS, with initially normal renal function at DPD
onset. Early recognition and diagnosis are therefore crucial to timely clinical intervention. The role of
prompt kidney biopsy and initiation of TPE and HD in PRS must not be underestimated. |
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| ISSN: | 0353-9466 1333-9451 |