Anti-GBM and anti-MPO antibodies coexist in a case of pulmonary renal syndrome

Hijosa, Marta Melgosa; Román, Laura Espinosa; Camblor, C. Fernández; García, María Luz Picazo; Salcedo, Dorotea Pascual; Torres, Marisa

doi:10.1007/s00467-004-1762-z

Cited by 15 publications

(3 citation statements)

References 13 publications

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“…Most cases of anti-GBM disease in pediatric patients have acute presentations with diffuse glomerular disease, including severe fibrinoid necrosis along with cellular crescent formation, with subsequent glomerular scarring leading to end-stage renal disease [6, 11, 20, 21, 22, 23, 24, 25]. More recent reports, where diagnosis and commencement of plasmapheresis has been prompt, have led to better outcomes [7, 26, 27, 28].…”

Section: Discussionmentioning

confidence: 99%

Anti-GBM disease after nephrectomy for xanthogranulomatous pyelonephritis in a patient expressing HLA DR15 major histocompatibility antigens: a case report

O’Hagan¹,

Mallett²,

Convery³

et al. 2015

CNCS

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Antiglomerular basement membrane (anti-GBM) antibody disease is uncommon in the pediatric population. There are no cases in the literature describing the development of anti-GBM disease following XGP or nephrectomy. We report the case of a 7-year-old boy with no past history of urological illness, treated with antimicrobials and nephrectomy for diffuse, unilateral xanthogranulomatous pyelonephritis (XGP). Renal function and ultrasound scan of the contralateral kidney postoperatively were normal. Three months later, the child represented in acute renal failure with rapidly progressive glomerulonephritis requiring hemodialysis. Renal biopsy showed severe crescentic glomerulonephritis with 95% of glomeruli demonstrating circumferential cellular crescents. Strong linear IgG staining of the glomerular basement membranes was present, in keeping with anti-GBM disease. Circulating anti-GBM antibodies were positive. Treatment with plasma exchange, methylprednisolone, and cyclophosphamide led to normalization of anti-GBM antibody titers. Frequency of hemodialysis was reduced as renal function improved, and he is currently independent of dialysis with estimated glomerular filtration rate 20.7 mls/min/1.73 m2. Case studies in the adult literature have reported the development of a rapidly progressive anti-GBM antibody-induced glomerulonephritis following renal surgery where patients expressed HLA DR2/HLA DR15 major histocompatibility (MHC) antigens. Of note, our patient also expresses the HLA DR15 MHC antigen.

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Section: Discussionmentioning

confidence: 99%

Anti-GBM disease after nephrectomy for xanthogranulomatous pyelonephritis in a patient expressing HLA DR15 major histocompatibility antigens: a case report

O’Hagan¹,

Mallett²,

Convery³

et al. 2015

CNCS

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“…The combination of DAH and acute or rapidly progressive (crescentic) glomerulonephritis, known as Goodpasture syndrome, is used interchangeably with PRS since several different pathogenic mechanisms underlie this clinical entity [16,22]. PRS most commonly results from AAV or anti-GBM disease but is also described in lupus, Henoch-Schönlein purpura, rheumathoid arthritis and other connective tissue diseases [2].…”

Section: Discussionmentioning

confidence: 99%

“…The outcome of reported pediatric cases is not different from that in adults: two patients died at presentation from massive pulmonary hemorrhage [14,18]; two patients, both dialysis dependent at presentation, remained in end-stage renal disease (ESRD) [6]; one patient, whose treatment was delayed 5 months after clinical onset, improved renal function [16]; three patients, including ours, had normal renal function several weeks to 10 months after onset of disease [15,17]. Our patient and a 12-year old girl reported by Paueksakon et al [15] had normal renal function at onset with cellular crescents in only 16% or 13% of glomeruli, respectively.…”

Section: Discussionmentioning

confidence: 99%

Pulmonary renal syndrome in a child with coexistence of anti-neutrophil cytoplasmic antibodies and anti-glomerular basement membrane disease: case report and literature review

et al. 2013

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BackgroundPulmonary renal syndrome (PRS), denoting the presence of diffuse alveolar hemorrhage and glomerulonephritis as manifestations of systemic autoimmune disease, is very rare in childhood. The coexistence of circulating anti-neutrophil cytoplasmic antibody (ANCA) and anti-glomerular basement membrane (GBM) disease in children affected by this syndrome is exceptional, with unfavorable outcome in five out of seven patients reported to date. We describe a child with PRS associated with both circulating anti-myeloperoxidase (anti-MPO) ANCA and anti-GBM disease on renal biopsy who was successfully treated with immunosuppressive therapy.Case presentationA 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.ConclusionWe 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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Pediatric double positive anti‐glomerular basement membrane antibody and anti‐neutrophil cytoplasmic antibody glomerulonephritis—A case report with review of literature

Helander

Hanna

Annen

2021

J of Clinical Apheresis

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Anti‐glomerular basement membrane (GBM) disease is a rarely described entity in the pediatric population, especially in those less than 3 years old. Even rarer, is double seropositive disease, consisting of anti‐GBM antibody plus anti‐neutrophil cytoplasmic antibodies. Both single and double antibody positive diseases are characterized by rapidly progressive glomerulonephritis, often without pulmonary involvement in the pediatric population. We report the case of a 2‐year‐old child with double seropositive anti‐GBM disease, the youngest in the current literature, along with the role of therapeutic plasma exchange and rituximab in disease treatment.

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Anti-GBM and anti-MPO antibodies coexist in a case of pulmonary renal syndrome

Cited by 15 publications

References 13 publications

Anti-GBM disease after nephrectomy for xanthogranulomatous pyelonephritis in a patient expressing HLA DR15 major histocompatibility antigens: a case report

Anti-GBM disease after nephrectomy for xanthogranulomatous pyelonephritis in a patient expressing HLA DR15 major histocompatibility antigens: a case report

Pulmonary renal syndrome in a child with coexistence of anti-neutrophil cytoplasmic antibodies and anti-glomerular basement membrane disease: case report and literature review

Pediatric double positive anti‐glomerular basement membrane antibody and anti‐neutrophil cytoplasmic antibody glomerulonephritis—A case report with review of literature

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