scholarly journals Case Report: Eculizumab and ECMO Rescue Therapy of Severe ARDS in Goodpasture Syndrome

2021 ◽  
Vol 8 ◽  
Author(s):  
Michael Sobotta ◽  
Onnen Moerer ◽  
Oliver Gross
Keyword(s):  
Pressure Support ◽  
Rescue Therapy ◽  
Pulmonary Hemorrhage ◽  
Organ Damage ◽  
Therapeutic Options ◽  
Lung Damage ◽  
Goodpasture's Syndrome ◽  
Goodpasture’S Syndrome ◽  
Steroid Pulse ◽  
Life Threatening

Introduction: Goodpasture's syndrome is a life-threatening autoimmune type IV collagen disease characterized by the presence anti–glomerular basement membrane antibodies, rapid progressive glomerulonephritis and/or pulmonary hemorrhage.Methods: Here, we describe new therapeutic options, which take recent advances in unraveling Goodpasture's pathogenesis into account.Results: In a 17-year old male, severe Goodpasture's syndrome resulted in acute respiratory distress syndrome (ARDS). Within 1 day after hospital admission, the patient required extracorporeal membrane oxygenation (ECMO). Despite steroid-pulse and plasmapheresis, ARDS further deteriorated. Eleven days after admission, the patient was in a pre-final stage. At last, we decided to block the complement-driven lung damage by Eculizumab. Three days after, lung-failure has stabilized in a way allowing us to initiate Cyclophosphamide-therapy. As mechanical ventilation further triggers Goodpasture-epitope exposure, the patient was taken from pressure support - breathing spontaneously by the help of maintaining ECMO therapy. After a total of 24 days, ECMO could be stopped and pulmonary function further recovered.Conclusions: In conclusion, our findings suggest that life-threatening organ-damage in Goodpasture's syndrome can be halted by Eculizumab as well as by lung-protective early withdrawal from pressure support by the help of ECMO. Both therapeutic options serve as new tools in otherwise hopeless situations to prevent further organ-damage and to gain time until the established immunosuppressive therapy works in otherwise lethal autoimmune-diseases.

scholarly journals Fcγ Receptor Iib–Deficient Mice Develop Goodpasture's Syndrome upon Immunization with Type IV Collagen

2000 ◽  
Vol 191 (5) ◽  
pp. 899-906 ◽  
Author(s):  
Akira Nakamura ◽  
Takae Yuasa ◽  
Azusa Ujike ◽  
Masao Ono ◽  
Toshihiro Nukiwa ◽  
...  
Keyword(s):  
Rapidly Progressive Glomerulonephritis ◽  
Pulmonary Hemorrhage ◽  
Basement Membranes ◽  
Collagen Type Iv ◽  
Goodpasture's Syndrome ◽  
Goodpasture’S Syndrome ◽  
Type Iv ◽  
Suitable Animal Model ◽  
And Function

The combination of hemorrhagic pneumonitis and rapidly progressive glomerulonephritis is a characteristic feature of Goodpasture's syndrome (GPS), an autoimmune disease resulting from the interaction of pathogenic anti–collagen type IV (C-IV) antibodies with alveolar and glomerular basement membranes. Lack of a suitable animal model for this fatal disease has hampered both a basic understanding of its etiology and the development of therapeutic strategies. We now report a novel model for GPS using mice deficient in a central regulatory receptor for immunoglobulin (Ig)G antibody expression and function, the type IIB Fc receptor for IgG (FcγRIIB). Mutant mice immunized with bovine C-IV reproducibly develop massive pulmonary hemorrhage with neutrophil and macrophage infiltration and crescentic glomerulonephritis. The distinctive linear, ribbon-like deposition of IgG immune complex seen in GPS was observed along the glomerular and tubulointerstitial membranes of diseased animals. These results highlight the role of FcγRIIB in maintaining tolerance and suggest that it may play a role in the pathogenesis of human GPS.

scholarly journals A case of Goodpasture's syndrome presenting with acute renal failure before pulmonary hemorrhage.

2001 ◽  
Vol 34 (8) ◽  
pp. 1205-1210
Author(s):  
Yoshiaki Nishioka ◽  
Yasuhide Kanamoto ◽  
Hiroshige Kawano ◽  
Masanobu Miyazaki ◽  
Takashi Harada ◽  
...  
Keyword(s):  
Renal Failure ◽  
Acute Renal Failure ◽  
Pulmonary Hemorrhage ◽  
Goodpasture's Syndrome ◽  
Goodpasture’S Syndrome

scholarly journals Impact of ANCA-Associated Vasculitis on Outcomes of Hospitalizations for Goodpasture’s Syndrome in the United States: Nationwide Inpatient Sample 2003–2014

Medicina ◽  
2020 ◽  
Vol 56 (3) ◽  
pp. 103 ◽  
Author(s):  
Charat Thongprayoon ◽  
Wisit Kaewput ◽  
Boonphiphop Boonpheng ◽  
Patompong Ungprasert ◽  
Tarun Bathini ◽  
...  
Keyword(s):  
Resource Utilization ◽  
The United States ◽  
Hospitalized Patients ◽  
Hospital Treatment ◽  
Goodpasture's Syndrome ◽  
Anca Vasculitis ◽  
Goodpasture’S Syndrome ◽  
Anca Associated Vasculitis ◽  
Significant Difference ◽  
Life Threatening

Background and objectives: Goodpasture’s syndrome (GS) is a rare, life-threatening autoimmune disease. Although the coexistence of anti-neutrophil cytoplasmic antibody (ANCA) with Goodpasture’s syndrome has been recognized, the impacts of ANCA vasculitis on mortality and resource utilization among patients with GS are unclear. Materials and Methods: We used the National Inpatient Sample to identify hospitalized patients with a principal diagnosis of GS from 2003 to 2014 in the database. The predictor of interest was the presence of ANCA-associated vasculitis. We tested the differences concerning in-hospital treatment and outcomes between GS patients with and without ANCA-associated vasculitis using logistic regression analysis with adjustment for other clinical characteristics. Results: A total of 964 patients were primarily admitted to hospital for GS. Of these, 84 (8.7%) had a concurrent diagnosis of ANCA-associated vasculitis. Hemoptysis was more prevalent in GS patients with ANCA-associated vasculitis. During hospitalization, GS patients with ANCA-associated required non-significantly more mechanical ventilation and non-invasive ventilation support, but non-significantly less renal replacement therapy and plasmapheresis than those with GS alone. There was no significant difference in in-hospital outcomes, including organ failure and mortality, between GS patients with and without ANCA-associated vasculitis. Conclusions: Our study demonstrated no significant differences between resource utilization and in-hospital mortality among hospitalized patients with coexistence of ANCA vasculitis and GS, compared to those with GS alone.

scholarly journals A case of Goodpasture's syndrome with massive pulmonary hemorrhage

2000 ◽  
Vol 15 (1) ◽  
pp. 99 ◽  
Author(s):  
Young Ok Kim ◽  
Jee Yeun Choi ◽  
Joon Il Park ◽  
Sun Ae Yoon ◽  
Chul Woo Yang ◽  
...  
Keyword(s):  
Pulmonary Hemorrhage ◽  
Goodpasture's Syndrome ◽  
Goodpasture’S Syndrome

scholarly journals Difficulties in Goodpasture's syndrome diagnosing

2019 ◽  
Vol 91 (3) ◽  
pp. 64-67 ◽  
Author(s):  
V I Podzolkov ◽  
G K Makhnach ◽  
T I Ishina ◽  
A B Ponomarev ◽  
I D Medvedev
Keyword(s):  
Basement Membrane ◽  
Rare Disease ◽  
Glomerular Basement Membrane ◽  
Clinical Case ◽  
Progressive Disease ◽  
Pulmonary Hemorrhage ◽  
Late Diagnosis ◽  
Diagnosis And Treatment ◽  
Goodpasture's Syndrome ◽  
Goodpasture’S Syndrome

The article analyzes the diagnosis and treatment of anti-GBM antibody disease (Goodpasture's syndrome) - a rare, severe progressive disease, associated with anti-glomerular basement membrane antibody-induced pulmonary hemorrhage and glomerulonephritis. The main problem of this pathology is late diagnosis, resulted in ineffective treatment. The article provides current information on the epidemiology, etiology and pathogenesis, diagnosis, and treatment of Goodpasture’s syndrome, as well as clinical case of a patient with this rare disease.

scholarly journals Goodpasture's syndrome: Case report

2004 ◽  
Vol 57 (7-8) ◽  
pp. 391-395
Author(s):  
Biljana Vuckovic ◽  
Tatjana Ilic ◽  
Igor Mitic ◽  
Violeta Knezevic ◽  
Slavenka Vodopivec ◽  
...  
Keyword(s):  
Case Report ◽  
Autoimmune Disease ◽  
Kidney Disease ◽  
Kidney Function ◽  
Kidney Biopsy ◽  
Pulmonary Hemorrhage ◽  
Laboratory Findings ◽  
Goodpasture's Syndrome ◽  
Important Thing ◽  
Goodpasture’S Syndrome

Introduction Goodpasture's syndrome is a rare, autoimmune disease characterized by pulmonary hemorrhage, glomerulonephritis and production of anti-GBM (glomerular basement membrane) antibodies. The etiology of this syndrome is still unknown. Goodpasture's syndrome usually starts with pulmonary hemorrhage, which is followed by symptoms of kidney disease. Laboratory findings often include: anemia, microhematuria, proteinuria, increased levels of urea and creatinine and anti-GBM antibodies. Diagnosis of this syndrome can be established by presence of pulmonary hemorrhage, pulmonary radiography, kidney biopsy and positive result of circulating anti-GBM antibodies. Treatment of this syndrome should be initiated as soon as possible using a combination of cortocosteroids, cytostatics and plasmapheresis. Case report The first symptoms in a nineteen-year-old female patient were caused by anemia. Two months later she reported symptoms of pulmonary hemorrhage. At that point of time she already had renal insufficiency and was immediately hospitalized. The same day we started therapy with cortocosteroids, endoxan and plasmapheresis was initiated. Recovery of pulmonary function was obtained, but kidney function was lost. Discussion and Conclusions The most important thing in regard to Goodpasture's syndrome is quick diagnosis. Because of that, if patients report any kind of pulmonary hemorrhage, this syndrome must be considered. At that point of time, kidney function is usually not irreversibly damaged. The second important thing in Goodpasture's syndrome is that treatment must be very aggressive using a combination of immunosuppressives and plasmapheresis. This is the only chance for these patients to avoid hemodialysis or death.

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