IgA-mediated anti-glomerular basement membrane disease. A case report (original) (raw)
Related papers
Anti-glomerular basement membrane disease
Kidney International, 2003
A 47-year-old white man who worked as a professional musician presented to his general practitioner with a 3-week history of malaise, loss of appetite, slight weight loss, and dark urine. There was no history of respiratory symptoms and, in particular, no hemoptysis. He had had mild asthma for 10 years, which had been treated with inhaled bronchodilators. He was a lifelong nonsmoker and drank alcohol only occasionally. He had no family history of renal disease. The general practitioner detected proteinuria and hematuria, and blood tests showed an elevated serum creatinine. The patient was referred to our renal unit the same day. On arrival, he looked well and was afebrile with no rash or edema. Pulse was 80 beats/minute and regular, and blood pressure was 145/70 mm Hg. Clinical examination of the heart, lungs, abdomen, and nervous system was normal. Urinalysis showed 2ϩ blood, 3ϩ protein, dysmorphic red cells, and granular and red cell casts. Serum creatinine was 550 mol/L (6.3 mg/dL) and urea 21 mmol/L. Electrolytes were in the normal range, but albumin was reduced at 2.3 g/dL. Blood picture showed hemoglobin 10.7 g/dL; white blood cell count 9.6 ϫ 10 9 /L; and platelets 654 ϫ 10 9 /L. Oxygen saturation with the patient breathing room air was 94%, and a chest radiograph was normal. An enzyme-linked immunosorbent assay (ELISA) for antiglomerular basement membrane (anti-GBM) antibodies was
Double positive Anti-GBM and ANCA-associated glomerulonephritis – A case report
2020
Double-Positive Anti-Glomerular Basement Membrane (Anti-GBM) and Anti-Neutrophil Cytoplasm Antibody (ANCA)- Associated Glomerulonephritis is a rare disease. The disease is characterized by concurrent presence of Anti-GBM antibodies and ANCA in a patient. The patient usually presents with rapidly progressive glomerulonephritis with or without pulmonary haemorrhage. We report a case of a middle-aged gentleman who presented acute kidney injury with a serum creatinine level of 459umol/L. He was tested positive for both Anti-GBM and ANCA with high titers. He underwent 6 cycles of plasma exchange, pulse IV cyclophosphamide and high dose steroid. During his 6 months outpatient review, a total of 6 doses IV cyclophosphamide has been given and his serum creatinine reduced to 146 umol/L. In conclusion, the use of pulse IV cyclophosphamide over oral form in this subject is empirical and showed marked improvement of renal function.
American Journal of Case Reports, 2015
Rare co-existance of disease or pathology Background: Anti-glomerular basement membrane disease (anti-GBM disease) is an autoimmune glomerulonephritis disease that is characterized by IgG linear deposition along the non-collagen domain of a3 chains of type IV collagen on the GBM. Although anti-GBM disease accompanied with IgA linear deposition along GBMs was discussed previously in some papers, anti-GBM disease combined with IgA granular deposition in the mesangial area, especially complicated with reversible posterior leukoencephalopathy syndrome (RPLS), was rarely reported. RPLS is usually caused by hypertensive encephalopathy, renal decompensation, fluid retention, and adverse effects of immunosuppressive drugs. Case Report: A male patient with the chief complaints of headache, gross hematuria, and nocturia was referred to our hospital. Based on renal biopsy, the diagnosis was finally confirmed as anti-GBM disease combined with IgA nephropathy and, the patient received comprehensive treatment, including cyclophosphamide (CTX), which led to symptom improvement. Two days after the third impulse CTX was given, he suddenly experienced headache and dizziness, which eventually developed into a tonic-clonic seizure. RPLS was identified by cranial magnetic resonance imaging (MRI) with reversible neuroimaging. After diazepam and antihypertension management, seizures were controlled. RPLS, a neurological complication, was found in anti-GBM disease with IgA nephropathy during our immunosuppressants therapy for the first time. Conclusions: It is worth paying more attention to patients with rapidly progressive glomerulonephritis (RPGN), as they might be complicated with RPLS during intravenous administration of CTX and methylprednisolone. We suggest the neuroimaging be examined as soon as the seizure happens.
Goodpasture’s Syndrome with Negative Anti-glomerular Basement Membrane Antibodies
European Journal of Case Reports in Internal Medicine, 2017
A young male patient with rapidly progressive and life-threatening pulmonary haemorrhage due to anti-glomerular basement membrane (anti-GBM) antibody disease without renal involvement repeatedly tested negative for serum anti-GBM antibodies. Although rare, anti-GBM antibody disease should be considered in the differential diagnosis in patients with life-threatening pulmonary haemorrhage due to isolated diffuse alveolar haemorrhage. Enzyme-linked-immunosorbent assay (ELISA) testing for anti-GBM antibodies in anti-GBM antibody disease can give false-negative results. A negative serum anti-GBM antibody test is therefore insufficient to exclude the diagnosis. Thus, a kidney or lung biopsy should be considered in any case with a high clinical suspicion but negative anti-GBM antibody test to confirm or rule out the diagnosis. LEARNING POINTS • Diffuse alveolar haemorrhage (DAH) is a life-threatening disorder caused by severe damage due to injury or inflammation of the alveolarcapillary basement membrane. • Anti-GBM antibody disease is a rare autoimmune disorder with circulating autoantibodies directed against the alpha-3 chain[Q2] of type VI collagen of the glomerular and/or alveolar basement membrane which may result in oliguric acute kidney failure due to rapidly progressive glomerulonephritis with or without DAH (commonly referred to as Goodpasture's syndrome). • A kidney or lung biopsy should be considered to confirm or rule out the diagnosis if there is a high clinical suspicion but the anti-GBM antibody test is negative; prompt diagnosis and initiation of plasmapheresis, cyclophosphamide and prednisone therapy is essential.
Antigenic Heterogeneity of IgA Anti-GBM Disease: New Renal Targets of IgA Autoantibodies
American Journal of Kidney Diseases, 2008
Anti-glomerular basement membrane (anti-GBM) disease is an aggressive form of glomerulonephritis, usually mediated by immunoglobulin G (IgG) autoantibodies to the noncollagenous (NC1) domain of ␣3(IV) collagen. Less is known about the target antigen(s) in patients with atypical anti-GBM disease involving IgA autoantibodies. We report a new case of IgA anti-GBM disease in a patient with a history of proliferative lupus nephritis who presented with increasing creatinine levels, proteinuria, and hematuria, but no clinical or serological evidence of lupus recurrence. Renal biopsy showed focal and segmental necrotizing glomerulonephritis with strong linear capillary loop IgA staining by means of immunofluorescence. Serological test results were negative for IgG or IgA autoantibodies against the ␣3NC1 domain. By means of immunoblotting, IgA from patient serum bound to 38-to 48-kd antigens collagenasesolubilized from human GBM, but not to purified NC1 domains of GBM collagen IV. The target of patient's IgA autoantibodies thus was identified as a novel GBM antigen, distinct from the ␣3NC1 domain or other known targets of anti-GBM IgA autoantibodies. Clinical resolution was attained by means of conventional treatment with steroids and cyclophosphamide. The diversity of antigens recognized by anti-GBM IgA autoantibodies highlights the importance of renal biopsy for the reliable diagnosis of this rare condition because conventional serological immunoassays likely would yield false-negative results. Am J Kidney Dis 52:761-765.
American Journal of Kidney Diseases, 2014
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´s disease. Anti-GBM antibodies are generally of immunoglobulin G subclass 1 (IgG1) and can in most cases readily be detected in the circulation using enzyme linked immunosorbent assays (ELISA). Here we report 4 cases where anti-GBM ELISA yielded negative or borderline results despite lifethreatening disease. All four patients were positive in IgG4 anti-GBM ELISA and all were ANCA negative. All cases were confirmed with kidney biopsy. Two of the patients exhibited higher results in anti-GBM ELISA when using a non-denaturing coating buffer. All four 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 only by IgG subclass specific tests or by kidney biopsy. * Sample sent to the reference laboratory at the time of the second kidney biopsy **Normal ranges in the assays are: total IgG <10; IgG1 <3; IgG2<8; IgG3 <27; IgG4<7 ELISA units. *** PLEX = plasma exchange, CYC iv = inter mitten intravenous cyclophosphamide, pred = prednisolone
An unusual case of anti-glomerular basement membrane disease presenting with nephrotic syndrome
International Urology and Nephrology
Anti-glomerular basement membrane (anti-GBM) disease is a vasculitic disease characterized by acute kidney injury, oliguria, hematuria and proteinuria. Proteinuria is rarely in the nephrotic range. A case of anti-GBM disease with proteinuria of 22.5g/day is discussed. Immunofluorescence showed strong linear IgG deposits while electron microscopy showed widespread visceral epithelial cell foot cell process effacement. No electron dense immune complex-type deposits were identified. Pathology findings were not suggestive of simultaneous presentation of anti-GBM disease and other diseases associated with nephrotic range proteinuria. Anti-GBM disease should be considered in a comprehensive differential diagnosis of severe proteinuria.