scholarly journals C3 glomerulonephritis associated with ANCA positivity: a case report

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Ling Li ◽  
Li-qin Liu ◽  
Ying-ying Yang ◽  
Zhang-Xue Hu
Keyword(s):  
Serum Creatinine ◽  
Membranous Nephropathy ◽  
Elevated Serum ◽  
Basement Membranes ◽  
Disease Classification ◽  
C3 Glomerulonephritis ◽  
Normal Range ◽  
Dense Deposit ◽  
Glomerular Lesions ◽  
Glomerular Deposits

Abstract Background C3 glomerulopathy (C3G) is a recent disease classification that is characterized by the presence of glomerular deposits (composed of C3) in the absence of significant amounts of immunoglobulin and comprises dense deposit disease and C3 glomerulonephritis (C3GN). Most C3GN manifests as membranoproliferative, mesangial proliferative glomerulonephritis patterns via light microscopy. Pure membranous nephropathy (MN)-like glomerular lesions are rare manifestations of C3GN. Anti-neutrophil cytoplasmic antibodies (ANCAs) are also seldomly reported to be positive in C3GN. Herein, we report the case of a C3GN patient presenting with an MN-like glomerular pattern with ANCA positivity. Case presentation A 68-year-old woman was admitted to a local hospital with elevated serum creatinine for two weeks. Laboratory tests showed a hemoglobin level of 85 g/L. Urinalysis was positive for 2 + protein and 360 RBCs/HPF. Blood biochemistry analysis revealed the following concentrations: albumin, 30.3 g/L; globulin, 46.2 g/L; blood urea nitrogen, 19.9 mmol/L; and serum creatinine, 234 µmol/L. The serum C3 level was 0.4950 g/L, and the serum C4 level was 0.1050 g/L. The direct Coombs test was positive. Serologic testing for ANCA revealed the presence of p-ANCA (1:10) by indirect immunofluorescence microscopy assay, as well as the presence of PR3 1.2 (normal range < 1) and MPO 3.5 (normal range < 1) by enzyme immunoassay. Renal biopsy sample pathology showed 2/6 cellular crescents and thickened glomerular basement membranes. Immunofluorescence testing revealed only diffuse, finely granular depositions of C3 along the glomerular capillary walls in frozen and paraffin-embedded tissue sections. Electron microscopy demonstrated the presence of subepithelial electron-dense deposits, similar to those that are observed in membranous nephropathy. Corticosteroid and cyclophosphamide were administered, with a subsequent improvement in renal function. Conclusions We present the rare case of a patient with MN-like C3GN with ANCA positivity. C3GN with ANCA positivity may be represented by more crescents, severe renal dysfunction and more extrarenal manifestations. More cases are needed to elucidate the clinicopathologic features and optimal treatments of these patients.

The Primary Glomerular Diseases

2017 ◽  
Author(s):  
Richard J. Glassock ◽  
Sanjeev Sethi ◽  
Fernando C. Fervenza
Keyword(s):  
Renal Biopsy ◽  
Membranous Nephropathy ◽  
Minimal Change Disease ◽  
Crescentic Glomerulonephritis ◽  
Minimal Change ◽  
C3 Glomerulonephritis ◽  
Focal And Segmental Glomerulosclerosis ◽  
Glomerular Diseases ◽  
Dense Deposit ◽  
Primary Glomerular Diseases

Glomerular disorders in which the manifestations of disease are primarily confined to the kidneys, without multisystem involvement, are not only common but very heterogeneous in terms of pathogenesis and clinical features. Typically, these primary glomerular diseases are characterized according to the findings on renal biopsy, as studied by light, immunofluorescence, and electron microscopy. The principal primary glomerular diseases are minimal change disease, focal and segmental glomerulosclerosis, membranous nephropathy, C3 glomerulonephritis and dense deposit disease, IgA nephropathy, and renal-limited crescentic glomerulonephritis. These clinicopathologic entities are discussed according to epidemiology, clinical features, pathology, pathogenesis (and genetics if appropriate), prognosis, and treatment, emphasizing recent findings.  Key words: C3 glomerulonephritis, dense deposit disease, focal and segmental glomerulosclerosis, glomerulonephritis, IgA nephropathy, membranous nephropathy, minimal change disease, renal biopsy, renal-limited crescentic glomerulonephritis

scholarly journals Membranous nephropathy associated with multicentric Castleman’s disease that was successfully treated with tocilizumab: a case report and review of the literature

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Ryosuke Saiki ◽  
Kan Katayama ◽  
Yosuke Hirabayashi ◽  
Keiko Oda ◽  
Mika Fujimoto ◽  
...  
Keyword(s):  
Membranous Nephropathy ◽  
Periodic Acid ◽  
Castleman’S Disease ◽  
Basement Membranes ◽  
The Body ◽  
Castleman's Disease ◽  
Multicentric Castleman’S Disease ◽  
High Zinc ◽  
Secondary Membranous Nephropathy ◽  
Multicentric Castleman's Disease

Abstract Background Multicentric Castleman’s disease is a life-threatening disorder involving a systemic inflammatory response and multiple organ failure caused by the overproduction of interleukin-6. Although renal complications of Castleman’s disease include AA amyloidosis, thrombotic microangiopathy, and membranoproliferative glomerulonephritis, membranous nephropathy is relatively rare. We experienced a case of secondary membranous nephropathy associated with Castleman’s disease. Case presentation The patient was a 43-year-old Japanese man who had shown a high zinc sulfate value in turbidity test, polyclonal hypergammaglobulinemia, anemia, and proteinuria. A physical examination revealed diffuse lymphadenopathy, an enlarged spleen and papulae of the body trunk. A skin biopsy of a papule on the patient’s back showed plasma cells in the perivascular area and he was diagnosed with multicentric Castleman’s disease, plasma cell variant. Kidney biopsy showed the appearance of bubbling in the glomerular basement membranes in Periodic acid methenamine silver stain and electron microscopy revealed electron dense deposits within and outside the glomerular basement membranes. Since immunofluorescence study showed predominant granular deposition of IgG1 and IgG2, he was diagnosed with secondary membranous nephropathy associated with Castleman’s disease. He was initially treated with prednisolone alone, however his biochemical abnormalities did not improve. After intravenous tocilizumab (700 mg every 2 weeks) was started, his C-reactive protein elevation, anemia, and polyclonal gammopathy improved. Furthermore, his urinary protein level declined from 1.58 g/gCr to 0.13 g/gCr. The prednisolone dose was gradually tapered, then discontinued. He has been stable without a recurrence of proteinuria for more than 6 months. Conclusions Tocilizumab might be a treatment option for secondary membranous nephropathy associated with Castleman’s disease.

MO427SAFE USE OF ALLOPURINOL TO TREAT ACUTE URIC ACID NEPHROPATHY IN PREGNANCY: A CASE REPORT

2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
Fahd Khan ◽  
Aizaz Ali ◽  
Jamie Willows ◽  
Didem Tez
Keyword(s):  
Uric Acid ◽  
Case Report ◽  
Serum Creatinine ◽  
Serum Uric Acid ◽  
Renal Tubules ◽  
Normal Range ◽  
History Of ◽  
Uric Acid Nephropathy ◽  
Acute Uric Acid Nephropathy ◽  
In Pregnancy

Abstract Introduction Acute uric acid nephropathy (UAN) is characterized by acute kidney injury (AKI) due to uric acid crystal precipitation within the distal tubules and collecting ducts. We present a young woman, with a history of hyperuricaemia, who was treated with allopurinol for acute UAN during her first pregnancy. She also continued allopurinol treatment during her second pregnancy for prevention of further acute UAN. To the author’s knowledge, this is the first case report of biopsy-confirmed acute UAN during pregnancy. Case report A 20 year old woman, who was 13 weeks pregnant, was admitted with AKI. Past medical history included chronic kidney disease (CKD) and gout since the age of 17. She had an extensive family history of CKD and gout (without diagnosis, despite genetic testing). She had been on daily allopurinol 300mg, but this was stopped 8 weeks prior by her rheumatology team due to concerns about teratogenicity. At that time serum creatinine was at her baseline of 100 μmol/L (normal range 50-120 μmol/L) and serum uric acid had been 740 μmol/L (normal range 140-360 μmol/L). On admission, she felt well and was euvolemic. Serum creatinine was now 352 μmol/L and her serum uric acid level was 1720 μmol/L, with an elevated urine uric acid to creatinine ratio of 1.1. She underwent renal biopsy, which showed significant deposition of uric acid crystals in the renal tubules, confirming a diagnosis of acute UAN. She was given intravenous fluids. The uncertainties of allopurinol use in pregnancy were discussed with her, and she was restarted on allopurinol 200 mg daily. Over the next 3 weeks, serum uric acid decreased to 470 μmol/L and serum creatinine to 116 μmol/L. She was maintained on allopurinol during her pregnancy and delivered a healthy baby girl. She was advised against further pregnancies due to increased risk of maternal and fetal complications. However, three years later she presented at 15 weeks’ gestation. After a discussion regarding the potential teratogenic effects of allopurinol versus the risk of recurrent severe AKI due to acute UAN if it was again discontinued, she chose to continue allopurinol. The pregnancy proceeded without complication. Her daughters are now 8 and 5 years old. They do not have any congenital malformations, though both have mild to moderate learning difficulties. Discussion Allopurinol is approved for the treatment of hyperuricaemia outside of pregnancy, but given it interrupts purine synthesis there is a biologically plausible concern regarding teratogenicity. However, in our patient with long-standing hyperuricaemia it was the discontinuation of allopurinol that precipitated AKI due to the resultant crystal formation when serum uric acid reached very high levels. Biopsy confirmation of acute UAN was vital in this case, given the possibility of missing an alternative diagnosis and the risks of giving empirical allopurinol therapy. Once the diagnosis for her severe AKI was confirmed, it was clear our patient would benefit from uric acid lowering therapy. Our patient had two healthy girls despite using allopurinol from week 16 in her first pregnancy and throughout her second pregnancy. Unfortunately, both girls have mild to moderate learning needs, though it is unprovable whether allopurinol was causative as no study has followed up long term outcomes after foetal exposure during pregnancy.

Abstract 16015: A Rare Cutaneous Adverse Drug Reaction With Apixaban

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Khezar Syed ◽  
Haseeb Chaudhary ◽  
Tayyab Ali Waheed ◽  
Ansar Aziz
Keyword(s):  
Serum Creatinine ◽  
Early Recognition ◽  
Atrial Fibrillation Patient ◽  
Skin Testing ◽  
Oral Anticoagulants ◽  
Elevated Serum ◽  
Cross Reactivity ◽  
Cell Counts ◽  
Macular Rash ◽  
Direct Acting

We report a case of apixaban-induced macular rash noticed 3 days after its initiation for atrial fibrillation. Patient denied any fever, arthralgias, myalgias or photosensitivity otherwise. On presentation, she complained of generalized pruritus with a bilateral non-blanchable macular rash on distal lower extremities and dorsum of feet. The labs showed normal cell counts, an elevated serum creatinine of 2.29mg/dl with a bland urine on microscopy. Apixaban was switched to LMWH. Serum creatinine returned to baseline with fluid therapy within 24 hours. Patient was re-challenged with apixaban that caused recurrence of generalized pruritus. It was treated successfully with diphenhydramine and prednisone. She was restarted on LMWH and rash disappeared after 3 days of stopping apixaban. She was later commenced on rivaroxaban without any complications. Several cutaneous adverse drug reactions (cADR) have been reported with direct acting oral anticoagulants (DOAC) like rivaroxaban and edoxaban but are extremely rare with apixaban. The overall reported incidence of dermatologic immune reactions with FXa inhibitors is <0.1%. Previously reported skin eruptions from apixaban include palmoplantar psoriasiform rash, leucocytoclastic vasculitis and acute generalized erythematous pustulosis. Our patient’s Naranjo scale was 7 and her rash improved after cessation of apixaban. The case illustrates a hypersensitivity reaction from apixaban that did not have cross-reactivity with other FXa inhibitors. Early recognition of cADR from this widely used DOAC can avoid potential complications. Minor reactions may be managed by switching to different DOAC therapy. Whether a cross-reactivity truly exists must be explored by validated skin testing.

scholarly journals Eculizumab for Dense Deposit Disease and C3 Glomerulonephritis

2012 ◽  
Vol 7 (5) ◽  
pp. 748-756 ◽  
Author(s):  
Andrew S. Bomback ◽  
Richard J. Smith ◽  
Gaetano R. Barile ◽  
Yuzhou Zhang ◽  
Eliot C. Heher ◽  
...  
Keyword(s):  
Dense Deposit Disease ◽  
C3 Glomerulonephritis ◽  
Dense Deposit

Falsely elevated serum creatinine concentration in ketoacidosis

1985 ◽  
Vol 107 (4) ◽  
pp. 562-564 ◽  
Author(s):  
Farahnak K. Assadi ◽  
Eunice G. John ◽  
Linda Fornell ◽  
Ira M. Rosenthal
Keyword(s):  
Serum Creatinine ◽  
Elevated Serum ◽  
Serum Creatinine Concentration ◽  
Creatinine Concentration ◽  
Elevated Serum Creatinine

Neural Epidermal Growth Factor–Like 1 Protein–Positive Membranous Nephropathy in Chinese Patients

2021 ◽  
pp. CJN.11860720
Author(s):  
Guoqin Wang ◽  
Lijun Sun ◽  
Hongrui Dong ◽  
Yanyan Wang ◽  
Xiaoyi Xu ◽  
...  
Keyword(s):  
Membranous Nephropathy ◽  
Clinical Characteristics ◽  
Chinese Patients ◽  
Double Positive ◽  
Immunohistochemistry Staining ◽  
Phospholipase A2 Receptor ◽  
Pathologic Features ◽  
Glomerular Deposits ◽  
Epidermal Growth

Background and objectivesThe neural EGF-like 1 (NELL-1) protein is a novel antigen in primary membranous nephropathy. The prevalence and clinical characteristics of NELL-1–positive membranous nephropathy in Chinese individuals with primary membranous nephropathy are unclear.Design, setting, participants, & measurementsA total of 832 consecutive patients with biopsy-proven primary membranous nephropathy were enrolled. The glomerular expression of phospholipase A2 receptor (PLA2R) and thrombospondin type 1 domain-containing 7A (THSD7A) was screened. Glomerular immunohistochemistry staining for NELL-1 was performed in 43 patients with PLA2R- and THSD7A-negative membranous nephropathy, 31 patients with PLA2R-positive membranous nephropathy, and two patients with PLA2R and THSD7A double positivity. The NELL-1 antibody was also detected in the sera of patients with NELL-1–positive membranous nephropathy by western blot. Clinical and pathologic features were comparable between patients with isolated NELL-1–positive, isolated PLA2R/THSD7A-positive, and triple antigen–negative membranous nephropathy.ResultsAmong the 832 patients with primary membranous nephropathy, 11 of 54 (20%) patients with PLA2R-negative membranous nephropathy had THSD7A-positive membranous nephropathy. NELL-1–positive membranous nephropathy accounted for 35% (15 of 43) of all patients with PLA2R- and THSD7A-negative membranous nephropathy. One patient was double positive for NELL-1 and PLA2R in glomerular deposits and positive for only the PLA2R antibody in the serum. Most patients with NELL-1–positive membranous nephropathy were women. No tumors were found. There were significant differences in the prevalence of IgG subtypes between patients with different antigen positivity. Among patients with isolated NELL-1–positive membranous nephropathy, although 80% (12 of 15) were IgG4 staining positive, the proportion of IgG4 dominance was only 67% (ten of 15).ConclusionsAbout one third of patients who were PLA2R and THSD7A negative were NELL-1 positive in Chinese patients with primary membranous nephropathy. NELL-1–positive membranous nephropathy was more common than THSD7A-positive membranous nephropathy in PLA2R-negative membranous nephropathy.

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