Abstract | BACKGROUND: CASE PRESENTATION: A 10-year old girl presented with fever, fatigue, malaise, and pallor followed by hemoptysis and severe anemia. Diffuse alveolar hemorrhage was revealed on fiberoptic bronchoscopy. Renal findings consisted of microscopic hematuria, moderate proteinuria, and anti-GBM disease on renal biopsy. ANCA with anti-MPO specificity were present whereas anti-GBM antibodies were on borderline for positivity. Methyl- prednisolone pulses followed by prednisone led to cessation of hemoptysis, marked improvement of lung fuction, and normal finding on chest x-ray within 10 days. An immunosuppressive regimen was then given consisting of prednisone daily for 4 weeks with subsequent taper on alternate day, i.v. cyclophosphamide pulses monthly for 6 doses, followed by mycophenolate mofetil that resulted in normal lung function tests, hemoglobin concentration, and anti-MPO level within four subsequent weeks. During 10-months of follow-up she remained well, her blood pressure and renal function tests were normal, and proteinuria and hematuria gradually resolved. CONCLUSION: We report a child with an exceptionally rare coexistence of circulating ANCA and anti-GBM disease manifesting as PRS in whom renal disease was not the prominent part of clinical presentation, contrary to other reported pediatric patients. A review of literature on disease with double positive antibodies is also presented. Evaluation of a patient with PRS should include testing for presence of different antibodies. An early diagnosis and rapid institution of aggressive immunosuppressive therapy can induce remission and preserve renal function. Renal prognosis depends on the extent of kidney injury at diagnosis and appropriate treatment.
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Authors | Radovan Bogdanović, Predrag Minić, Jasmina Marković-Lipkovski, Nataša Stajić, Nataša Savić, Milan Rodić |
Journal | BMC nephrology
(BMC Nephrol)
Vol. 14
Pg. 66
(Mar 22 2013)
ISSN: 1471-2369 [Electronic] England |
PMID | 23517575
(Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't, Review)
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Chemical References |
- Antibodies, Antineutrophil Cytoplasmic
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Topics |
- Anti-Glomerular Basement Membrane Disease
(blood, complications, diagnosis)
- Antibodies, Antineutrophil Cytoplasmic
(blood)
- Child
- Female
- Glomerulonephritis
(blood, complications, diagnosis)
- Hemorrhage
(blood, complications, diagnosis)
- Humans
- Lung Diseases
(blood, complications, diagnosis)
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