Case 52-1993

Cabot, Richard C.; Scully, Robert E.; McNeely, William F.; McNeely, Betty U.; Fanburg, Barry L.; Niles, John L.; Mark, Eugene J.

doi:10.1056/nejm199312303292708

Cited by 17 publications

(5 citation statements)

References 35 publications

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“…Several pathogenetic hypothesis have been proposed to explain this coexistence, the most attractive one suggests an ANCA development as the initial event, with ANCA-associated mechanisms leading to the exposure of the otherwise hidden α-3(IV)NC1 antigen and triggering anti-GBM antibody production [11,23,28]. This hypothesis is supported by the reports on sequential positivity of ANCA followed by anti-GBM antibodies [26,29], by the strong association between elevated ANCA titers and anti-GBM disease [14,19], and by the recent findings that anti-PR3 and/or anti-MPO antibodies were always detected long before anti-GBM antibodies in anti-GBM disease patients [30]. However, the opposite sequence in double positive patients is also described [31-33].…”

Section: Discussionmentioning

confidence: 97%

“…Searching PubMed, we were able to retrieve only seven pediatric cases reported to date, either within case series [6,18] or as single case reports [14-17]. Their main features together with those in our patient are presented in Table 1.…”

Section: Discussionmentioning

confidence: 99%

“…However, we could not find a similar case among AAV patients or double positive patients reported to date. The unfavorable outcome in children with double positivity and DAH, having either low or high percentage of glomeruli with crescents [6,14,18], strongly remind of the serious nature of the disease for which we do not still have robust indicator to assist with optimal personalized therapy.…”

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%

“…Their coexistence (double or dual positivity) was found in a subset of adult patients at disease presentation indicating a pathogenic link [6-13]. In children, this coexistence is extremely rare with only seven patients reported to date, five of them had an unfavorable outcome [6,14-18]. …”

Section: Introductionmentioning

confidence: 99%

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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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Section: Discussionmentioning

confidence: 97%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

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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Pulmonary renal syndrome in childhood: A report of twenty-one cases and a review of the literature

et al. 2000

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In adults, the term specific pulmonary renal syndrome describes disorders with pulmonary and glomerular manifestations and includes Wegener's granulomatosis, Goodpasture disease, and systemic lupus erythematosus. Nonspecific pulmonary renal syndrome refers to either pulmonary disease complicating glomerular disease, or glomerular diseases following pulmonary disease. Since little is known regarding pulmonary renal syndrome in childhood, we reviewed the charts of 21 pediatric patients with pulmonary renal syndromes treated by the Department of Pediatrics, University of Bern between 1991 and 1998; we also reviewed the pediatric literature that deals with specific pulmonary renal syndromes. Specific pulmonary renal syndrome was noted in 3 children with systemic vasculitis (Wegener granulomatosis, N = 2; microscopic polyangiitis, N = 1) and 2 with systemic lupus erythematosus. Nonspecific pulmonary renal syndrome was observed in 12 patients with pulmonary edema (N = 9), pulmonary thromboembolism (N = 2), and pulmonary infection (N = 1) complicating the course of a glomerular disease, and in 4 children with a pulmonary disease followed by a glomerular disease. Review of the literature disclosed 52 cases of specific pulmonary renal syndrome other than systemic lupus erythematosus: Wegener granulomatosis (N = 28), Goodpasture disease (N = 13), and Henoch‐Schönlein purpura (N = 11). In addition, hemolytic uremic syndrome complicated pneumococcal pneumonia in 32 cases. We conclude that pulmonary renal syndromes need to be looked for in childhood. Apart from Wegener granulomatosis, Goodpasture disease, and systemic lupus erythematosus, Henoch‐Schönlein purpura and hemolytic‐uremic syndrome occasionally have both pulmonary and renal features. Pediatr Pulmonol. 2000; 29:382–388. © 2000 Wiley‐Liss, Inc.

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ARDS und Wegener-Granulomatose

et al. 1997

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A 19-year-old female patient had developed acute respiratory and renal failure after a prolonged period (many months) of antibiotic resistant otitis, sinusitis and mastoiditis. The patient had required intubation at another hospital and there was a history of tension pneumothorax and cardiopulmonary resuscitation during mechanical ventilation. Emergency extracorporeal membrane oxygenation (ECMO) for acute hypercapnic and hypoxic respiratory failure was instituted and the patient was transported to our institution while on ECMO. The patient was treated empirically for suspected pulmonary and systemic infection and received hydrocortisone (0.18 mg/kg/h) as part of a protocol-driven treatment of septic shock in addition to antibiotic and antimycotic regime. The use of ECMO was required for 10 and mechanical ventilation for another 50 days after admission. After successful extubation, central nervous system dysfunction became evident with a somnolent and generally unresponsive patient. When the hydrocortisone dose was gradually tapered, the clinical status of the patient further deteriorated, pulmonary gas exchange worsened and she developed renal failure with proteinura and hematuria. A renal biopsy was performed demonstrating vasculitis and focal segmental glomerulonephritis, a systemic granulomatous vasculitis was suspected; the serum was tested for anti-proteinase 3 antibodies (PR3-ANCA) and turned out to be positive (17.5 U/ml; normal range < 7 U/ml). The morphologic findings from renal biopsy, the positive test for antiproteinase 3 antibodies and the pulmonary-renal involvement with evidence of multisystem disease established the diagnosis of Wegener's granulomatosis. Immunosuppressive therapy with cyclophosphamide and prednisolone was instituted resulting in rapid improvement with recovery of pulmonary, renal and central nervous system function within two weeks. The use of ECMO in this patient served as a life-saving immediate measure usefull to "buy time" until a definite diagnosis could be established. ARDS represents an uniform pulmonary reaction to a large number of different noxious stimuli and disease entities. This case demonstrates that intensive care physicians caring for critically ill patients with ARDS should include even rare causes of pulmonary injury into their differential diagnosis.

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Case 52-1993

Cited by 17 publications

References 35 publications

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

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

Pulmonary renal syndrome in childhood: A report of twenty-one cases and a review of the literature

ARDS und Wegener-Granulomatose

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