Goodpasture’s syndrome with absence of circulating anti-glomerular basement membrane antibodies: a case report

Fernandes, Rui Aniceto Nascimento; Freitas, Sara; Cunha, Pedro; Alves, Glória da Anunciação; Cotter, Jorge

doi:10.1186/s13256-016-0984-6

Cited by 11 publications

(7 citation statements)

References 14 publications

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“…The kidney biopsy shows the fibrinoid necrosis. The research shows that early and aggressive therapy leads to improved disease forecasts (5). Patients may be twice as positive for anti-GBM and anti-proteinase-3 neotrophili cytoplasmic antibodies (c-ANCA).…”

Section: Discussionmentioning

confidence: 99%

Lost kidney in Goodpasture syndrome: Case report

Kovačević

Janićijević

Petrovic

2020

Sanamed

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Introduction: Goodpasture syndrome is a rare autoimmune syndrome with alveolar hemorrhages and glomerulonephritis caused by circulating antibodies against the glomerular-basement-membrane. Anti-glomerular-basement-membrane were administered against a non-collagen (NC-1) a3 chain of collagen type IV, which was found at the highest concentration in the basal membrane of renal and pulmonary capillaries. The aim of case report is a clinical overview of this rare and severe syndrome.Case report: The patient, 22-years-old was sent to Center of Urgent Medicine of Clinical Center Kragujevac from General Hospital of Para}in because of blood poisoning, fever, symptoms and signs of renal and respiratory weakness, and suspected of Wegener's disease. Antibodies were taken on the basement membrane of the glomerulus were resulted of enormously high. After due to the clinical and immunology diagnosis of Goodpasture syndrome, plasmapheresis treatment was initiated. The standard hemodialysis was continued. Following the guidelines protocols, patient received pulse dozes of cytostatics, corticosteroids, etc. Conclusion:The case report of our patient points to the necessity of multidisciplinary approach of the expert team, consisting of a nephrologist, pulmologist, clinical pharmacologist and other specialists. The prognosis is good, if treatment is started before irreversible pulmonary and/or renal changes (respiratory and/or renal insufficiency). Goodpasture syndrome often progresses rapidly, so it can be fatal if it's delayed with the diagnosis and the treatment. Patients with Goodpasture's syndrome require an adequate the socio-medical care as a rare and severe syndrome.

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Section: Discussionmentioning

confidence: 99%

Lost kidney in Goodpasture syndrome: Case report

Kovačević

Janićijević

Petrovic

2020

Sanamed

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“…It is hypothesized that the structural component (NC-1) a-3 of type IV collagen chains, which is present in the basement membranes of the pulmonary and renal capillaries, plays the role of an autoantigen. Formed antibodies against the components of the basement membranes of the alveoli and renal glomeruli (anti-GBM antibodies) in the presence of C3-complement bind to antigens; the resulting immune complexes are deposited along the basement membranes, inducing immuno-inflammatory damage to the renal glomeruli (glomerulonephritis) and pulmonary alveoli (alveolitis) [7,9,11]. In the development of autoimmune inflammation, activation of cellular elements (T-lymphocytes, endotheliocytes, monocytes, alveolar macrophages, polymorphonuclear leukocytes), cytokines (insulin-like, platelet growth factors, tumor necrosis factors, interleukin-1), free radicals, proteolytic enzymes, adhesive molecules and other factors damaging renal tissue, play a significant role [10].…”

Section: The Goodpasture Syndrome Is An Immune-inflammatory Pathologymentioning

confidence: 99%

Goodpasture Syndrome: Case Report

Старченко

Vynnyk

Sovhyria

et al. 2019

Med. and Ecol. probl.

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The Goodpasture syndrome is an immune-inflammatory pathology characterized by the formation of autoantibodies directed against the basement membranes of the renal glomeruli and pulmonary alveoli, manifested by hemorrhagic pneumonitis in combination with pulmonary hemorrhage (hemoptysis) and glomerulonephritis. To date, etiological mechanisms of the disease are unknown. Clinical observations indicate a relationship between the development of Goodpasture syndrome and viral infection, intake of medications, industrial hazards. The incidence of Goodpasture syndrome is estimated to be 1 case per 1 million population. Due to the rarity of this pathology, each case of Goodpasture syndrome is of great theoretical and practical interest. The authors conducted the analysis of clinical and morphological observation of Goodpasture syndrome. The postmortem study revealed morphological signs indicating the presence of Goodpasture syndrome inter vivos, whose main manifestations were hemorrhagic pneumonitis and mesangial-proliferative glomerulonephritis with fibroplastic transformation. The reported case is also of particular interest in terms of correct formulation of the final diagnosis, since there was a combination of two diseases: Goodpasture syndrome and phlegmonous appendicitis with focal peritonitis. In this case, pulmonary heart failure should be considered as the direct cause of death, since pulmonary lesions prevailed over the renal ones, which is fully consistent with the clinical presentation and findings of the laboratory tests. The diagnosis of Goodpasture syndrome made at the hospital is not always timely, as can be evidenced by patient’s severe condition, and it requires careful differentiation with a number of other diseases involving hemorrhagic pulmonary and renal syndrome, and the urgent need for active therapy with immunosuppressants, including prednisone and cytostatics. The timely adequate treatment significantly improves the prognosis.

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“…[3] The diagnosis of anti-GBM disease is typically made by immunofluorescence (IF) for IgG (IgG1 dominant) demonstrating linear staining of glomerular basement membranes and focal linear staining of tubular basement membranes, and the serologic presence of anti-GBM antibodies [1, 2, 4, 5]. Recently, atypical cases of anti-GBM disease characterized by negative serology for circulating anti-GBM antibodies and unusual histology have been reported [6–12].…”

Section: Introductionmentioning

confidence: 99%

A case of aggressive atypical anti-GBM disease complicated by CMV pneumonitis

et al. 2019

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BackgroundAnti-glomerular basement membrane (anti-GBM) disease is characterized by circulating IgG glomerular basement membrane antibodies and is clinically expressed as a rapidly progressive crescentic glomerulonephritis (GN), with 30–60% of patients also developing pulmonary hemorrhage. Classically, the renal biopsy shows glomerular crescent formation, bright linear staining of glomerular basement membranes (GBM) for IgG on direct immunofluorescence (IF), and the serologic presence of circulating anti-GBM antibodies. Recently, patients with linear IgG IF staining, undetectable circulating anti-GBM antibodies and glomerular changes atypical for anti-GBM disease have been described as “atypical anti-GBM disease”, with a distinctly more benign clinical course than typical anti-GBM disease. We present a case report of a patient with negative anti-GBM serology but positive linear IgG staining by IF, severe diffuse crescentic and endocapillary proliferative glomerulonephritis, and renal failure, complicated by severe pulmonary hemorrhage after immunosuppression, likely due to cytomegalovirus (CMV) pneumonitis.Case presentationA 24-year-old man was admitted to hospital with hemoptysis and renal failure. Investigations for anti-GBM serology by addressable laser bead immunoassay (ALBIA) was negative for anti-GBM antibodies. Renal biopsy showed diffuse endocapillary proliferative glomerulonephritis with membranoproliferative features and diffuse circumferential crescents. Direct IF showed strong linear staining for IgG along GBMs. The patient’s hemoptysis improved with immunosuppression, but 1 month later he was readmitted with gross hemoptysis, which was refractory to further cyclophosphamide, plasma exchange and rituximab. Bronchoalveolar lavage (BAL) and blood work confirmed CMV pneumonitis, and the patient’s hemoptysis resolved with ganciclovir, though he became dialysis dependent.ConclusionsThis case demonstrates an atypical presentation of anti-GBM disease with both crescents and endocapillary hypercellularity and negative serology. The patient is dialysis dependent, unlike most previously described patients with atypical anti-GBM disease. The course was complicated by CMV pneumonitis, which contributed to the severity of the pulmonary manifestations and added diagnostic difficulty.

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Goodpasture’s syndrome with absence of circulating anti-glomerular basement membrane antibodies: a case report

Cited by 11 publications

References 14 publications

Lost kidney in Goodpasture syndrome: Case report

Lost kidney in Goodpasture syndrome: Case report

Goodpasture Syndrome: Case Report

A case of aggressive atypical anti-GBM disease complicated by CMV pneumonitis

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