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      Veno-venous extracorporeal membrane oxygenation (VV-ECMO) for life-threatening isolated pulmonary anti-GBM disease

      case-report

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          Abstract

          Anti-glomerular basement membrane disease (anti-GBM disease) associated with renal and lung lesions has a poor prognosis. Diffuse alveolar hemorrhage (DAH) is a complication that worsens anti-GBM disease prognosis. We report a rescue case using veno-venous extracorporeal membrane oxygenation (VV-ECMO) for diffuse alveolar hemorrhage due to isolated pulmonary anti-GBM disease; a rare anti-GBM syndrome. A 30-year-old Japanese female with no past medical history. Presented with acute hypoxemic respiratory failure requiring mechanical ventilation. Progressive deterioration and refractory hypoxemia prompted therapy with VV-ECMO. Serum anti-GBM antibody confirmed the diagnosis of anti-GBM disease. Multi-modal systemic therapy with pulse-dosed methylprednisolone, plasma exchange, and rituximab resulted in significant clinical improvement. VV-ECMO for 10 days was uncomplicated. Renal replacement therapy was not required. The patient was extubated on day 18 and discharged from the hospital after 45 days. VV-ECMO supportive therapy for DAH with refractory respiratory failure was demonstrated to be effective pending definitive diagnostic and therapeutic management in this case of isolated pulmonary anti-GBM disease.

          Highlights

          • •We report a rescue case using VV-ECMO for diffuse alveolar hemorrhage with isolated pulmonary anti-GBM disease.

          • •VV-ECMO can provide life-support bridge therapy until a response to disease control with fundamental specific therapy becomes effective.

          • VV-ECMO with associated required anticoagulation was an effective and safe life-support therapy in the setting of DAH.

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          Most cited references11

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          Clinical features and outcome of patients with both ANCA and anti-GBM antibodies.

          Patients have been described who have both anti-neutrophil cytoplasm antibodies (ANCA) and anti-glomerular basement membrane (GBM) antibodies. We have attempted to define the true prevalence of such "double positive" patients, and describe in detail their clinical features and outcome. We have reviewed all serologic assays performed between 1990 and 2000 in a single institution, and the case notes of patients having sera positive for both ANCA and anti-GBM antibodies. During this time 20,392 sera were initially tested for ANCA, and 4808 sera tested for anti-GBM antibodies. Five percent of all ANCA-positive serum samples were also positive for anti-GBM antibodies, and 32% of all anti-GBM positive samples had detectable ANCA. Of 27 patients with both antibodies, 82% had anti-myeloperoxidase specific P-ANCA. Pulmonary hemorrhage occurred in 44%. Renal biopsy showed extensive glomerular cellular crescents in most patients. Patient and renal survival rates were 52% and 26%, respectively, at one year. Sixty-eight percent of patients were dialysis-dependent at presentation, and none of these recovered renal function, despite immunosuppression with or without plasma exchange. Serologic evidence of double positivity for both ANCA and anti-GBM antibodies is common in patients with either antibody. In our study these patients have a poor prognosis when presenting with severe disease and initially behave more like anti-GBM disease than vasculitis. Recovery from severe renal failure is rare.
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            Extracorporeal membrane oxygenation in the management of diffuse alveolar hemorrhage.

            Extracorporeal membrane oxygenation (ECMO) may be used to support patients with severe hypoxemic respiratory failure refractory to conventional mechanical ventilation. However, because systemic anticoagulation is generally required to maintain circuit patency, severe bleeding is often seen as a contraindication to ECMO. We describe our center's experience with four patients who received ECMO for refractory hypoxemic respiratory failure due to diffuse alveolar hemorrhage (DAH), a condition for which anticoagulation is typically contraindicated, and provide a review of the literature. The mean age was 35.8 ± 16.4 years. The mean pre-ECMO PaO2 to FIO2 ratio was 52.3 ± 9.4 mm Hg. All patients were treated with continuous infusions of heparin with a goal-activated partial thromboplastin time between 40 and 60 seconds (mean, 47.4 ± 11.6 seconds). All four subjects (100%) survived to decannulation, and three subjects (75%) survived to discharge. The results from this case series, along with previously published data, suggest that ECMO is a reasonable management option for patients with DAH-associated severe, refractory hypoxemic respiratory failure. This is especially true in the era of modern ECMO technology where lower levels of anticoagulation are able to maintain circuit patency while minimizing bleeding risk.
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              Circulating anti-glomerular basement membrane antibodies with predominance of subclass IgG4 and false-negative immunoassay test results in anti-glomerular basement membrane disease.

              Autoantibodies against a constituent of the glomerular basement membrane (GBM), the α3-chain of type IV collagen, can cause both rapidly progressive glomerulonephritis and alveolar hemorrhage, referred to as anti-GBM disease or Goodpasture disease. Anti-GBM antibodies generally are of immunoglobulin G subclass 1 (IgG1) and can in most cases readily be detected in the circulation using enzyme-linked immunosorbent assays (ELISAs). We report 4 cases in which anti-GBM ELISA yielded negative or borderline results despite life-threatening disease. All 4 patients had positive results by IgG4 anti-GBM ELISA and all had undetectable antineutrophil cytoplasmic antibody. All cases were confirmed with kidney biopsy. Two of the patients showed higher signal in anti-GBM ELISA when using a nondenaturing coating buffer. All 4 were young women with severe alveolar hemorrhage and favorable renal outcome, suggesting that patients with predominance of IgG4 autoantibodies may constitute a distinct subgroup of anti-GBM disease. We conclude that patients with idiopathic alveolar hemorrhage can have anti-GBM disease detected by only IgG subclass-specific tests or kidney biopsy. Copyright © 2014 National Kidney Foundation, Inc. Published by Elsevier Inc. All rights reserved.
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                Author and article information

                Contributors
                Journal
                Respir Med Case Rep
                Respir Med Case Rep
                Respiratory Medicine Case Reports
                Elsevier
                2213-0071
                01 June 2022
                2022
                01 June 2022
                : 38
                : 101680
                Affiliations
                [a ]Acute and Critical Care Center, Department of Acute and Critical Care Medicine, Sapporo Higashi Tokushukai Hospital, 3-1, N33 E14, Higashi-ku, Sapporo City, Hokkaido Prefecture, 065-0033, Japan
                [b ]John A Burns School of Medicine, 651 Llalo St., Honolulu, HI, 96816, USA
                Author notes
                []Corresponding author. support@ 123456higashi-tokushukai.or.jp
                Article
                S2213-0071(22)00102-2 101680
                10.1016/j.rmcr.2022.101680
                9168115
                c74e38a3-3697-49a0-a6ad-91fdc2adffd6
                © 2022 Published by Elsevier Ltd.

                This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

                History
                : 2 April 2022
                : 18 May 2022
                : 25 May 2022
                Categories
                Case Report

                isolated pulmonary anti-gbm disease,diffuse alveolar hemorrhage (dah),veno-venous extracorporeal membrane oxygenation (vv-ecmo),vv-ecmo, veno-venous extracorporeal membrane oxygeneation,anti-gbm disease, anti-glomerular basement membrane disease,dah, diffuse alveolar hemorrhage,bal, brochoalveolar lavage,hit, heparin-induced thrombocytopenia,er, emergency room

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