scholarly journals Atypical anti-glomerular basement membrane disease with anti-GBM antibody negativity and ANCA positivity: a case report

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Na Guo ◽  
Qinghua Yin ◽  
Song Lei ◽  
Yanjun He ◽  
Ping Fu
Keyword(s):  
Basement Membrane ◽  
Glomerular Basement Membrane ◽  
Rare Case ◽  
Test Results ◽  
Serum Samples ◽  
Tissue Samples ◽  
Case Presentation ◽  
Severe Renal Insufficiency ◽  
Laboratory Test Results ◽  
Organ Specific

Abstract Background Anti-glomerular basement membrane (anti-GBM) disease is an organ-specific autoimmune disease that involves the lung and kidneys and leads to rapid glomerulonephritis progression, with or without diffuse alveolar hemorrhage, and even respiratory failure. Classic cases of anti-GBM disease are diagnosed based on the presence of the anti-GBM antibody in serum samples and kidney or lung biopsy tissue samples. However, atypical cases of anti-GBM disease are also seen in clinical practice. Case presentation We herein report the rare case of a patient with atypical anti-GBM disease whose serum was negative for the anti-GBM antibody but positive for the myeloperoxidase (MPO) anti-neutrophil cytoplasmic antibody (p-ANCA) and another atypical ANCA. Laboratory test results showed severe renal insufficiency with a creatinine level of 385 μmol/L. Renal biopsy specimen analysis revealed 100% glomeruli with crescents; immunofluorescence showed immunoglobulin G (IgG) linearly deposited alongside the GBM. Finally, the patient was discharged successfully after treatment with plasmapheresis, methylprednisolone and prednisone. Conclusion This patient, whose serum was negative for the anti-GBM antibody but positive for p-ANCA and another atypical ANCA, had a rare case of anti-GBM disease. Insights from this unusual case might help physicians diagnose rare forms of glomerulonephritis and treat affected patients in a timely manner.

scholarly journals Rare case of atypical crescentic glomerulonephritis and interstitial lung disease with negative anti-GBM antibody and positive anti-MPO antibody

2019 ◽  
Vol 12 (8) ◽  
pp. e229256 ◽  
Author(s):  
Alexander Hanna ◽  
Jenny Ross ◽  
Fernanda Heitor
Keyword(s):  
Hepatitis B ◽  
Basement Membrane ◽  
Glomerular Basement Membrane ◽  
Rare Case ◽  
Crescentic Glomerulonephritis ◽  
Kidney Biopsy ◽  
Flank Pain ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Hepatitis B Infection ◽  
Double Positive

A 70-year-old man presented with 1 month of haematuria and mild right-sided flank pain with no other symptoms. Diagnostic workup included serum studies which showed the presence of antimyeloperoxidase antibodies, a kidney biopsy which demonstrated necrotising crescentic glomerulonephritis with linear immunofluorescence of the basement membrane, and electron microscopy which exhibited thickening of the glomerular basement membrane. Incidentally, the patient was discovered to have a latent hepatitis B infection, which complicated immunosuppressive therapy. He was treated with a course of plasmapheresis and methylprednisolone, followed by entecavir for hepatitis B prophylaxis, and finally by rituximab. This case of glomerulonephritis was notable for its resemblance to the better known Goodpasture’s disease. Typically, Goodpasture’s syndrome exists on a spectrum from seronegative disease to double-positive disease that presents with both anti–glomerular basement membrane (anti-GBM) and cytoplasmic-antineutrophil cytoplasmic antibodies/antiproteinase 3 antibodies (c-ANCA/anti-PR3). However, this patient’s glomerulonephritis was unique because he presented negative for anti-GBM antibodies and positive for perinuclear-antineutrophil cytoplasmic antibodies/antimyeloperoxidase antibodies (p-ANCA/anti-MPO).

scholarly journals A Rare Cause of Acute Abdomen: An Isolated Falciform Ligament Necrosis

2014 ◽  
Vol 2014 ◽  
pp. 1-3 ◽  
Author(s):  
Ziya Taner Ozkececı ◽  
Mustafa Ozsoy ◽  
Bahadır Celep ◽  
Ahmet Bal ◽  
Coskun Polat
Keyword(s):  
Acute Abdomen ◽  
Leukocyte Count ◽  
Exploratory Laparotomy ◽  
Falciform Ligament ◽  
Test Results ◽  
Imaging Studies ◽  
Anatomical Structures ◽  
Case Presentation ◽  
Air Density ◽  
Laboratory Test Results

The falciform ligament is one of the anatomical structures which attach the liver to the diaphragm and anterior abdominal wall. Primary falciform ligament is very rare. In this article, we present a case of an isolated falciform ligament necrosis, a rare primary pathology of the falciform ligament, who was admitted with acute abdomen. Case presentation: A 64-year-old female patient was admitted with the complaints of pain. Laboratory test results showed a leukocyte count of 17,000/mm3. Imaging studies demonstrated intra-abdominal reactionary fluid along with a heterogeneous mass localized in the falciform ligament. Exploratory laparotomy revealed a necrotic mass of the falciform ligament. No other pathology responsible for falciform ligament necrosis was found. We believe that falciform ligament necrosis should be considered a preliminary diagnosis, if any ligament abnormality, tumor, intraligament air density, or the presence of reactionary fluid surrounding the ligament is detected through abdominal imaging studies.

scholarly journals The development of rapidly progressive glomerulonephritis associated with both antineutrophil cytoplasmic antibody-associated vasculitis and anti-glomerular basement membrane nephritis in the course of nontuberculous mycobacterium infection: a case report

2020 ◽  
Vol 4 (1) ◽  
Author(s):  
Mikiya Kato ◽  
Risa Wakiya ◽  
Tomohiro Kameda ◽  
Kousuke Inoue ◽  
Tadashi Sofue ◽  
...  
Keyword(s):  
Basement Membrane ◽  
Glomerular Basement Membrane ◽  
Rapidly Progressive Glomerulonephritis ◽  
Mycobacterial Infection ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Nontuberculous Mycobacterium ◽  
Case Presentation ◽  
One Year ◽  
Sequential Appearance

Abstract Background Antineutrophil cytoplasmic antibodies (ANCA) and Anti-glomerular basement membrane (GBM) antibodies often induce rapidly progressive glomerulonephritis (RPGN). Some reports have demonstrated RPGN with the sequential appearance of ANCA then anti-GBM antibodies, suggesting that ANCA may induce the development of anti-GBM antibodies. Whereas, many reports have shown that the development of ANCA is associated with various infectious diseases, such as non-tuberculous mycobacterial infection. Case presentation A 65-year-old woman with pulmonary non-tuberculous mycobacterial (NTM) infection was monitored without treatment. One year later, serum myeloperoxidase (MPO)- ANCA were elevated (14.1 U/mL (normal value < 3.0 U/ml)). A high fever and RPGN appeared 1 year later, and serum MPO-ANCAs were 94.1 U/mL. Anti-GBM antibodies were also detected. A renal biopsy revealed crescentic glomerulonephritis with linear deposits of IgG and C3c along the GBM and interstitial inflammation with endarteritis of arterioles. The diagnosis was RPGN associated with anti-GBM nephritis and ANCA-associated vasculitis. Conclusion This report shows that preceding NTM infection may have induced ANCA and anti-GBM antibodies and caused the development of RPGN.

Studies of anionic sites of the kidney by re-injection of polyethyleneimine (PEI) after disappearance of PEI-particles by initial administration

1990 ◽  
Vol 48 (3) ◽  
pp. 742-743
Author(s):  
Kiyoko Nakono ◽  
Ashio Yoshimura ◽  
Hiroaki Oniki ◽  
Terukuni Ideura ◽  
Shozo Koshikawa
Keyword(s):  
Molecular Weight ◽  
Basement Membrane ◽  
Intravenous Injection ◽  
Glomerular Basement Membrane ◽  
Left Kidney ◽  
Renal Tissue ◽  
Anionic Sites ◽  
Tissue Samples ◽  
Group A ◽  
Group B

As anionic sites of glomerular basement membrane (GBM) stained by intravenous injection of PEI became obscure 2 hours after administration, we studied reappearance of the anionic sites upon re-injection of PEI.The following two experimental groups were prepared: In group A; 10 rats were injected with 0.3ml of 0.5% PEI (MW=1,800) solution, and left nephrectomy was performed after 3 hours (group A-1, N=5) and 5 hours (group A-2, N=5). Each tissue samples was subjected to contrasting/fixation immersion, washing, post-fixation and staining. Immediately after nephrectomy, re-administration of 0.3ml of the same PEI solution was performed on each animal. Right nephrectomy was done 15 minutes after re-injection of PEI, and renal tissue was treated the same way as that of the left kidney (group A-1', group A-2'). In group B; PEI with a molecular weight of 70,000 was used. Injection of PEI solution, nephrectomy and treatment of renal tissues were carried out in the same way as for group A (group B-l and B-l', N=6; group B-2 and B-2', N=8).

scholarly journals Cold agglutinin-induced hemolytic anemia during room temperature fluid resuscitation: a case report

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Yosuke Kawai ◽  
Miyoshi Deguchi ◽  
Naoko Mizouchi ◽  
Satoru Yoshida ◽  
Ken Kumagai ◽  
...  
Keyword(s):  
Hemolytic Anemia ◽  
Fluid Resuscitation ◽  
Room Temperature ◽  
Cold Agglutinin ◽  
Test Results ◽  
Cold Agglutinin Disease ◽  
Cell Agglutination ◽  
Case Presentation ◽  
Laboratory Test Results ◽  
Old Japanese

Abstract Background Cold agglutinin disease can cause the agglutination of red blood cells and hemolytic anemia due to cold temperature. Herein, we report a case of progressive hemolytic anemia due to cold agglutinin disease during fluid resuscitation and in the absence of exposure to cold. Case presentation A 71-year-old Japanese man was admitted to the emergency department with signs of hypotension and disturbed consciousness. He was diagnosed with diabetic ketoacidosis, and treatment with fluid resuscitation and insulin infusion was initiated. Laboratory test results obtained the following day indicated hemolytic anemia. On day 5 after admission, red blood cell agglutination was detected, and the patient was diagnosed with cold agglutinin disease. Conclusions Cold agglutinin disease should be considered in the differential diagnosis of progressive hemolytic anemia during fluid resuscitation, even if the solution is at room temperature.

scholarly journals Antigen-specific radioimmunoassays for anti-neutrophil cytoplasmic antibodies in the diagnosis of rapidly progressive glomerulonephritis.

1991 ◽  
Vol 2 (1) ◽  
pp. 27-36
Author(s):  
J L Niles ◽  
G L Pan ◽  
A B Collins ◽  
T Shannon ◽  
S Skates ◽  
...  
Keyword(s):  
Basement Membrane ◽  
Renal Disease ◽  
Glomerular Basement Membrane ◽  
Crescentic Glomerulonephritis ◽  
Rapidly Progressive Glomerulonephritis ◽  
Diagnostic Value ◽  
Proteinase 3 ◽  
Serum Samples ◽  
Additional Control ◽  
Pathologic Findings

Circulating anti-neutrophil cytoplasmic antibodies (ANCA) have been described in most patients with "pauci-immune" necrotizing and crescentic glomerulonephritis. A 29-kDa serine protease (p29 or proteinase 3) and myeloperoxidase are the two best characterized antigens recognized by ANCA. The study presented here was conducted to define the diagnostic value of assays for antibodies against these two antigens in rapidly progressive glomerulonephritis. Radioimmunoassays were developed for anti-p29 and anti-myeloperoxidase antibodies, with purified antigens, and the results of the radioimmunoassays were compared with those obtained by immunofluorescence tests for ANCA. We performed assays on serum samples from 123 patients with the syndrome of rapidly progressive glomerulonephritis, as well as from 200 blood bank donors and from 717 additional control patients. Without knowledge of the results of ANCA tests, the renal pathologic findings in the 123 patients with rapidly progressive glomerulonephritis were analyzed, and 42 were classified as pauci-immune necrotizing and crescentic glomerulonephritis, 18 were classified as anti-glomerular basement membrane nephritis and 63 were classified as other forms of renal disease. We found radioimmunoassays to be more reliable in the diagnosis of pauci-immune necrotizing and crescentic glomerulonephritis than immunofluorescence testing. By radioimmunoassay, ANCA were found in 40 of 42 patients (95% sensitivity) with pauci-immune necrotizing and crescentic glomerulonephritis (14 with anti-p29 and 26 with anti-myeloperoxidase antibodies). The tests for antibodies to p29 and myeloperoxidase were 99.9 and 99.5% specific for pauci-immune necrotizing and crescentic glomerulonephritis, respectively. In the setting of rapidly progressive glomerulonephritis, a positive radioimmunoassay for anti-p29 or anti-myeloperoxidase antibodies (together with a negative test for anti-GBM antibodies) gives a probability of pauci-immune necrotizing and crescentic glomerulonephritis of over 99%.

scholarly journals The Heparanase Inhibitor (Sulodexide) Decreases Urine Glycosaminoglycan Excretion and Mitigates Functional and Histological Renal Damages in Diabetic Rats

2019 ◽  
Vol 46 (2) ◽  
pp. 41-46
Author(s):  
S. Roshan-Milani ◽  
J. Khalilpour ◽  
A. Abdollahzade Fard
Keyword(s):  
Basement Membrane ◽  
Glomerular Basement Membrane ◽  
Wistar Rats ◽  
Diabetic Rats ◽  
Creatinine Ratio ◽  
Pathological Changes ◽  
Urine Protein ◽  
Tissue Samples ◽  
Protein Excretion ◽  
Male Wistar Rats

Abstract Background/objectives: Recent data suggest a role for heparanase in several proteinuric conditions. An increased glomerular heparanase expression is associated with loss of heparan sulfate in the glomerular basement membrane (GBM). The aim of the present study was to investigate the renal effects of heparanase inhibition in a diabetic experimental model. Methods: Fifteen male Wistar rats (230 ± 20 g) were divided into three groups: 1) controls, 2) diabetics (STZ, 50 mg/kg, dissolved in saline, ip), 3) diabetics + heparanase inhibitor (Sulodexide 1/5 mg/kg per day, gavage). The treatment started on the 21st day, for 21 consecutive days. The rats were kept individually in a metabolic cage (8 AM-2 PM) and urine samples were collected on the 21st and 42nd day. At study end blood, urine and tissue samples were collected for biochemical (blood BUN and Cr, urine GAG and Protein) and histological analyses. Results: The results of this study showed that the heparanase inhibitor (sulodexide) significantly decreased urine GAG and protein excretion, urine protein/creatinine ratio and serum BUN and Cr in streptozotocin-induced DN in the rats. Pathological changes were significantly alleviated in the DN rats having received the heparanase inhibitor (sulodexide). Conclusion: Our data suggest that the heparanase inhibitor (sulodexide) is able to protect against functional and histopathological injury in DN.

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