Unusual Case of Posterior Reversible Encephalopathy Syndrome in a Patient with Anti-glomerular Basement Membrane Antibody Glomerulonephritis: A Case Report and Review of the Literature

Posterior reversible encephalopathy syndrome (PRES) is characterized by a clinical and radiological entity with the sudden onset of seizures, headache, altered consciousness, and visual disturbances in patients with the findings of reversible vasogenic subcortical edema without infarction. Hypertension, renal disease, and autoimmune disease are co-morbid conditions of PRES. Nevertheless, there have only been a few case reports of PRES in a patient with anti-glomerular basement membrane antibody glomerulonephritis (anti-GBM GN). This paper presents the possible first Korean case of a 36-year-old woman with the striking features of PRES. She presented with a sudden onset of visual blindness, headache, and seizure. The brain MRI images revealed hyperintense lesions in both the occipital and parietal lobes, which suggested vasogenic edema. Three months before this presentation, she was diagnosed with anti-GBM GN. Since then, she underwent immunosuppression with cyclophosphamide and steroid, and hemodialysis for renal failure with a treatment of anti-GBM GN.

A Case of Crescentic Glomerulonephritis with Coexisting Anti-Glomerular Basement Membrane Antibodies and Myeloperoxidase-Anti-Neutrophil Cytoplasmic Autoantibodies / 대한내과학회지

Rapidly progressive glomerulonephritis (RPGN) is a clinical syndrome involving abrupt or insidious onset of hematuria, proteinuria, and anemia, and rapidly progressive renal failure. Crescentic glomerulonephritis is a histopathological term for RPGN showing extensive extracapillary proliferation, i.e., crescent formation. There are three major immunopathological categories of crescentic glomerulonephritis: anti-glomerular basement membrane (anti-GBM) antibody disease, immune complex-mediated, and pauci-immune (anti-neutrophil cytoplasmic autoantibody [ANCA]-positive). A small minority of all patients with glomerulonephritis develop crescentic glomerulonephritis. Anti-GBM antibodies and ANCA rarely coexist. There have been a few reports of dual positive crescentic glomerulonephritis with anti-GBM antibodies and ANCA in Korea. Here, we describe the case of a 73-year-old woman showing RPGN clinically and crescentic glomerulonephritis pathologically with coexisting anti-GBM antibodies and myeloperoxidase-ANCA.

A Case of P-ANCA-Positive Anti-Glomerular Basement Membrane Antibody Disease / 대한내과학회지

Up to 40% of patients with anti-glomerular basement membrane (GBM) disease, which is a rare autoimmune disorder usually manifesting as rapidly progressive glomerulonephritis (RPGN), are positive for circulating anti-neutrophil cytoplasmic antibody (ANCA). Many previous reports showed poor outcomes in these "double-positive" patients. We report a patient with perinuclear (p)-ANCA positive anti-GBM disease who presented with RPGN and required hemodialysis. Plasmapheresis and steroid and cyclophosphamide therapy were initiated following renal biopsy and resulted in normalization of anti-GBM antibody and p-ANCA titers, recovery of renal function, and discontinuation of hemodialysis. This case suggests that aggressive immunosuppression with plasmapheresis in patients who are p-ANCA positive with anti-GBM disease should be considered, even in those with severe renal dysfunction.

Two Cases of Anti-Glomerular Basement Membrane Antibody Disease Associated with Perinuclear-Anti-Neutrophil Cytoplasmic Antibody / 대한내과학회지

Anti-glomerular basement membrane antibody (anti-GBM Ab) disease is characterized by circulating antibodies to the glomerular basement membrane and the deposition of IgG or, rarely, IgA along the glomerular basement membrane. This disease accounts for 10-20% of crescentic glomerulonephritis. We report two patients with anti-GBM Ab disease who were positive for perinuclear-anti-neutrophil cytoplasmic antibody (p-ANCA). Percutaneous renal biopsies showed many crescent formations and linear deposits of IgG along the glomerular basement membrane. Serologic tests for p-ANCA were positive. They were treated with steroid pulse and cyclophosphamide and one patient also underwent plasma exchange therapy. Despite immunosuppressive therapy, their renal functions did not improve and both required regular hemodialysis.

Analysis of clinicopathology and plasmapheresis efficacy in patients with anti-glomerular basement membrane disease / 中华肾脏病杂志

objective To analyze the clinicopathological features and prognosis of antiglomerular basement membrane(GBM)disease,and evaluate the efficacy and safety of double filtration plasmapheresis(DFPP). Methods A total of 35 hospitalized patients diagnosed as anti-GBM disease in our department were enrolled in the study.All the patients were divided into 3 groups according to the manifestations at admission.Group Ⅰ∶24 patients with severe pulmonary hemorrhage or rapidly progressive glomerulonephritis(RPGN)received pulse methylprednisolone with or without DFPP,and then followed by prednisone and CTX.Group Ⅱ∶5 patients without severe pulmonary hemorrhage and RPGN received prednisone and CTX.Group Ⅲ∶5 ESRD patients and 1 normal renal function patient did not receive immunosuppression therapy.Anti-GBM antibody titer of pre-and post-DFPP in 4 patients was measured consecutively,and removal rate was calculated.Results The mean age of all the patients was(41.1±16.6)years.Sixteen patients(45.7%)presented Goodpasture's syndrome.Eighteen patients(51.4%)had anti-GBM glomerulonephritis alone,whereas one suffered solely from pulmonary hemorrhage.20%patients had positive P-ANCA serology.54.2%crescentic glomerulonephritis and 7 with other glomerulonephritis were revealed by kidney biopsy in 24 patients.Patients in Group Ⅰ showed more severe manifestation at admission:higher Scr level,higher titer of anit-GBM antibody,greater percentage of crescents.Within the follow-up period,7 patients died and kidneys of 50%patients survived.No patient died in Group Ⅱ and Ⅲ.The elder age,anemia,higher Scr(＞300 μmol/L),oliguria or anuria,emergency hemodialysis at admission,and more glomerular sclerosis were predictors of poor prognosis.The anti-GBM antibody was negative after 4 to 6 sessions of DFPP.and the mean removal rate was 55%.During total 94 DFPP sessions,there was no unacceptable morbidity. Conclusions Different therapy strategy is necessary for anti-GBM disease with different clinical manifestations.DFPP is an effective and safe clearance way of anti-GBM antibody.

Screening and identification of neutralizated single-chain antibody of anti-glomerular basement membrane antibody / 中华微生物学和免疫学杂志

Objective To screen a human single-chain variable fragments(scFv)against antiGBM antibody.Methods Using phage display technique,the phage antibody library was panned by antiglomerular basement membrane(GBM)antibody which was coated in a micro-titer plate,one clone was found to have high affinity to anti-GBM antibody.The DNA sequence of the positive clone was determined.Results Along with the increase of rounds anti-GBM antibody specific phage antibody was highly enriched and screening efficiency was increased 137 folds than the firest round.ELISA and competition inhibition assay showed that the scFv had a specific combination character with anti-GBM antibody.DNA sequencing confirmed that the whole gene of scFv was 750 bp,and in accordance with humanized single-chain variable region antibody sequence structure.Conclusion The results suggested that the scFv fragment to anti-GBM antibody could be successfully selected by recombinant phage antibody technique,which will laid an experimental foundation for further research of the therapy of Goodpasture syndrome.

A Case of Idiopathic Membranous Nephropathy with Crescents Formation Associated with Anti-GBM Antibody / 대한신장학회지

Crescents formation associated with anti-glomerular basement membrane (anti-GBM) antibody is a rare complication in patients with idiopathic membranous nephropathy (MN). Here, we report a 25-year-old male with crescentic MN associated with anti-GBM antibody, who presented with general edema and decreased renal function. Renal biopsy specimen revealed thickening of the glomerular capillary wall consistent with MN. Of 12 glomeruli, three sclerosed, and two crescents formations with interstitial fibrosis were seen. Serology was positive for anti-GBM antibody with titer of 125 enzyme unit (EU)/mL. The patient was treated with pulse intravenous methylprednisolone for 3 days followed by oral prednisolone, and intravenous cyclophosphamide. His renal function was improved significantly 2 weeks after initiation of treatment. In conclusion, crescents formation should be considered in patients with nephrotic syndrome accompanied with acute renal dysfunction, and the early biopsy of the kidney and appropriate treatment will be required to improve the clinical outcome.

A Case of Goodpasture's Syndrome / 대한신장학회잡지

Goodpasture's syndrome is an autoimmune disease characterized by rapid progressive glomerulonephritis, alveolar hemorrhage and autoantibody to the NC1 domain of the alpha 3 chain of type IV collagen (anti-glomerular basement membrane antibody). It was common in Europe with peak incidence at third decades. However, Goodpasture's syndrome was reported only three cases in Korea. We reported a case of Goodpasture's syndrome in a 34 year old male. He admitted our hospital due to dyspnea and hemoptysis. We made diagnosis by detection of Anti-GBM Ab and biopsy of kidney. He was treated with hemodialysis, plasma exchanges and immunosuppressive agents. However, his renal function was not recovered. Currently, he is receiving regular hemodialysis.

A Case of Anti-Glomerular Basement Membrane Antibody Disease without Pulmonary Hemorrhage / 대한신장학회잡지

Anti-glomerular basement membrane antibody mediated rapidly progressive glomerulonephritis is a rare autoimmune disease. It is characterized by acuterenal failure and crescentic glomeruli with linear immune deposits along glomerular basement membrane mediated by anti-GBM antibodies. We report a case of a sixty-years-old man with generalized edema and hematuria. On admission, BUN/Creatinine was 118/19.6 mg/dL, Hb was 10.2 g/dL. On urinalysis, protein was 3+, and many RBCs were found. Renal biopsy specimen which contained 8 glomeruli showed active cellular crescent formation in all glomeruli. On immunofluorescent staining specimen, there were 4 glomeruli which showed strong IgG linear staining along the glomerular basement membrane and mild C3 & C1q deposit along the capillary walls. The titer of anti-GBM antibody was 123 EU by ELISA (normal: <10 EU). We treated with high dose of corticosteroid and plasmapheresis, but renal function was not recovered even after 3 months of hemodialysis.

A Case of Rapidly Progressive Glomerulonephitis Associated with Anti-Glomerular Basement Membrane Antibody and p-Antineutrophilic Cytoplasmic Antibody / 대한신장학회잡지

Rapidly progressive glomerulonephritis(RPGN) is clinical syndrome characterized by rapid loss of renal function within several weeks to months, with histologic finding of extensive crescent formation. We report a case of RPGN associated with anti-glomerular basement membrane antibody(anti-GBM Ab) and perinuclear-antineutrophilic cytoplasmic antibody(p- ANCA), which rapidly progressed to chronic renal failure. A 44-year-old male was referred to our hospital for evaluation of pitting edema and proteinuria. Both anti-GBM Ab and p-ANCA were detected in serum. Percutaneous renal biopsy showed many crescents with some fibrinoid materials and heavy deposits of IgG. He was treated with pulse methylprednisolone, followed by oral corticosteroid and cyclophosphamide. In spite of immunosuppressive therapy, his renal function deteriorated rapidly and uremic symptoms including pulmonary edema were aggravated. He was started on hemodialysis and he has received regular hemodialysis without recovery of renal function. Further studies will be needed to determine the clinical significance of combined anti- GBM Ab and ANCA.

A case of Goodpasture syndrome with diffuse pulmonary hemorrhage / 대한내과학회지

Goodpasture syndrome is an autoimmune disease with a triad of acute renal failure due to rapidly progressive glomerulonephritis (RPGN), pulmonay hemorrhage and circulating anti-glomerular basement membrane antibody (anti-GBM Ab). It was commonly reported from Europe in male with a peak incidence in their 20's. If patients are affected with the disease, relief of symptoms can be expected by eliminating the anti-GBM Ab from the circulatory system through hemodialysis, plasmapheresis and immunoadsorption. However, if the diagnosis or treatment is delayed, the patients usually die from massive pulmonary hemorrhage. It has been revealed that the main target of anti-GBM Ab's is NC1 domain on the alpha3 chain of type IV collagen. Currently there are many studies underway using this information as a basis to identify the pathogenesis of Goodpasture syndrome and to develop new therapeutic approach. The patient was a 20-year-old male with a chief complaint of edema. Unlike patients in the two previous cases, reported in Korea who had massive hemorrhage, he showed diffuse pulmonary hemorrhage which improved in only one week by hemodialysis. Renal biopsy demonstrated crescents in over 90% of glomeruli and showed signs of acute renal failure due to RPGN, with 618 U/mL (normal range <19.9 U/mL) of anti-GBM Ab titer.

A Case of Anti-glomerular Basement Membrane Antibody mediated Rapidly Progressive Glomerulonephritis / 대한신장학회잡지

Anti-glomerular basement membrane antibody mediated rapidly progressive glomerulonephritis(anti- GBM antibody mediated RPGN) is defined by the clinical picture of renal failure developing over days or weeks and the histological appearance of crescents and linear immune deposits mediated by the circulating autoantibodies. We report a case of anti-GBM antibody mediated RPGN with review of literature. A 59-year-old female patient was admitted to the Chungang Gil Hospital because of fever and acute deterioration of renal function. On admission, hemoglobin was 7.39g/dL, hematocrit was 20.9%, and BUN/Cr were 39.7 and 5.23mg/dL respectively. Urinalysis showed albumin (1+) with many RBCs. Renal biopsy revealed the presence of segmental or circumferential cellular crescents associated with smooth linear staining of glomerular basement membrane with antibody to IgG. High titer of circulating antibody to glomerular basement membrane antigen was demonstrated by the ELISA. High doses of corticosteroid with plasmapheresis were administered, but her renal function was progressively deteriorated.