SP099DISTRIBUTION OF GLOMERULAR DEPOSITS OF IGG SUBCLASSES IN PARAFFIN EMBEDDED RENAL BIOPSY SPECIMENS OF LUPUS NEPHRITIS AND IDIOPATHIC MEMBRANOUS NEPHROPATHY

Membranous nephropathy is one of the most common causes of nephrotic syndrome in the adult population. According to the underlying etiology, membranous nephropathy is classified as either primary or secondary. Systemic lupus erythematosus is an autoimmune disease that can affect the kidneys in 50% of patients in the course of the disease. Renal disease may be the first manifestation of systemic lupus erythematosus and the development of systemic findings may be delayed for about 1–5 years following the diagnosis of lupus nephritis. We present a 59-year-old male patient who had a diagnosis of idiopathic membranous nephropathy since 2007 and developed membranous lupus nephritis during the 12-year follow-up without any extrarenal systemic lupus erythematosus findings.

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Clinical and pathological features of idiopathic membranous nephropathy with focal segmental sclerosis

10.21203/rs.2.12239/v2 ◽

2019 ◽

Author(s):

jiatong li ◽

Bing Chen ◽

Caifeng Gao ◽

Jing Huang ◽

Yongmei Wang ◽

...

Keyword(s):

Risk Factor ◽

Renal Biopsy ◽

Membranous Nephropathy ◽

Independent Risk Factor ◽

Urinary Protein ◽

Idiopathic Membranous Nephropathy ◽

Lesion Group ◽

Pathological Features ◽

Focal Segmental Sclerosis ◽

Clinical And Pathological Features

Abstract Background Our goal was to investigate the clinical and pathological features and prognosis of idiopathic membranous nephropathy (IMN) with focal segmental lesions. Methods In our hospital, 305 patients with nephrotic syndrome confirmed by renal biopsy as IMN were divided into a non-focal segmental lesion group (FSGS- group) and a focal segmental glomerulosclerosis (FSGS) group (FSGS+ group) and retrospectively analyzed. A total of 180 patients were followed for periods ranging from six months to two years. The general clinical and pathological data of both groups were compared, and the effects of different treatment schemes on the prognosis of both groups were observed. Results The FSGS+ group had a longer disease course, higher blood pressure levels, and higher serum creatinine and β 2 -microglobulin levels than the FSGS- group (all P < 0.05). Pathologically, the FSGS+ group had increased glomerular sclerosis, glomerular mesangial hyperplasia, acute tubular lesion and chronic tubular lesion rates (all P< 0.05). The remission rate was lower in the FSGS+ group than in the FSGS- group (64.7% vs 82.2%) and was lower in patients treated with calmodulin inhibitors than in those treated with cyclophosphamide in the FSGS+ group (P < 0.01). Survival analysis showed that the FSGS+ group had a poor prognosis (χ 2 =4.377，P=0.036). Risk factor analysis suggested that age at renal biopsy (P=0.006), 24-hour urinary protein quantity (P=0.01), chronic tubulointerstitial lesions (P=0.055), and FSGS lesions (P= 0.062) were risk factors for worsening renal condition; 24-hour urinary protein quantity was an independent risk factor for worsening renal condition. Conclusions Membranous nephropathy with FSGS is a risk factor, but not an independent risk factor, for IMN. Patients with membranous nephropathy with FSGS often present hypertension and tubule injury. The nonselective drug cyclophosphamide is preferred, and calcineurin inhibitors should be used with caution. Key words Idiopathic membranous nephropathy; focal segmental sclerosis; cyclophosphamide; calmodulin inhibitor; prognosis;

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M-Type Phospholipase A2 Receptor Staining in Children with Idiopathic Membranous Nephropathy: PLA2R Staining in Children with IMN

The Open Urology & Nephrology Journal ◽

10.2174/1874303x01912010027 ◽

2019 ◽

Vol 12 (1) ◽

pp. 27-32

Author(s):

Yosuke Inaguma ◽

Atsutoshi Shiratori ◽

Taku Nakagawa ◽

Kyoko Kanda ◽

Makiko Yoshida ◽

...

Keyword(s):

Phospholipase A2 ◽

Renal Biopsy ◽

Clinical Data ◽

Membranous Nephropathy ◽

Pediatric Patients ◽

Idiopathic Membranous Nephropathy ◽

Target Antigen ◽

Positive Staining ◽

Phospholipase A2 Receptor

Background: Membranous Nephropathy (MN) is a common cause of nephrotic syndrome in adults that can also occur in children, albeit less frequently. Recently, the M-type phospholipase A2 receptor (PLA2R) was identified as the target antigen in idiopathic membranous nephropathy (IMN), making it a useful marker for diagnosis. However, there are few studies describing the potential role of PLA2R in children with IMN. The aim of this study was to clarify the involvement of PLA2R in childhood IMN. Methods: We enrolled 11 patients diagnosed with IMN from January 1998 to March 2017. We performed PLA2R staining in paraffin-embedded renal biopsy sections. The clinical data were collected from the patients’ medical records. Results: The median age at biopsy was 6 years (range, 4 to 14 years). A single 6-year-old boy among all pediatric patients with IMN had granular PLA2R staining along his glomerular capillary loops and the prevalence of PLA2R-positivity was 9%. He also showed IgG4 co-dominant staining in terms of IgG subclass. There were no apparent differences in his clinical features such as clinical data at the time of renal biopsy, the time from the treatment initiation to remission, and relapse or renal dysfunction during the follow-up period. Conclusion: We suggest that PLA2R staining can be a diagnostic tool for patients with IMN of any age, though pediatric patients with IMN have lower prevalence of PLA2R-positive staining than adult patients.

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Pauci-Immune Necrotizing and Crescentic Glomerulonephritis with Membranous Lupus Nephritis, Fifteen Years after Initial Diagnosis of Secondary Membranous Nephropathy

Case Reports in Nephrology ◽

10.1155/2015/120762 ◽

2015 ◽

Vol 2015 ◽

pp. 1-5 ◽

Cited By ~ 1

Author(s):

Ryan Burkhart ◽

Nina Shah ◽

Michael Abel ◽

James D. Oliver ◽

Matthew Lewin

Keyword(s):

Lupus Nephritis ◽

Renal Biopsy ◽

Immune Complex ◽

Lupus Erythematosus ◽

Membranous Nephropathy ◽

Crescentic Glomerulonephritis ◽

Renal Involvement ◽

Kidney Injury ◽

Rapid Decline ◽

Secondary Membranous Nephropathy

Renal involvement in systemic lupus erythematosus (SLE) is usually immune complex mediated and may have multiple different presentations. Pauci-immune necrotizing and crescentic glomerulonephritis (NCGN) refers to extensive glomerular inflammation with few or no immune deposits that may result in rapid decline in renal function. We report a case of a 79-year-old Hispanic male with a history of secondary membranous nephropathy (diagnosed by renal biopsy 15 years previously) who was admitted with acute kidney injury and active urinary sediment. P-ANCA titers and anti-myeloperoxidase antibodies were positive. The renal biopsy was diagnostic for NCGN superimposed on a secondary membranous nephropathy. A previous diagnosis of SLE based on American College of Rheumatology criteria was discovered via Veteran’s Administration records review after the completion of treatment for pauci-immune NCGN. ANCAs are detected in 20–31% of patients with SLE. There may be an association between SLE and ANCA seropositivity. In patients with lupus nephritis and biopsy findings of necrotizing and crescentic glomerulonephritis, without significant immune complex deposition, ANCA testing should be performed. In patients with secondary membranous nephropathy SLE should be excluded.

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IgG subclasses in patients with membranoproliferative glomerulonephritis, membranous nephropathy, and lupus nephritis

Kidney International ◽

10.1038/ki.1997.32 ◽

1997 ◽

Vol 51 (1) ◽

pp. 270-276 ◽

Cited By ~ 136

Author(s):

Hirokazu Imai ◽

Keiko Hamai ◽

Atsushi Komatsuda ◽

Hiroshi Ohtani ◽

Akira B. Miura

Keyword(s):

Lupus Nephritis ◽

Membranous Nephropathy ◽

Membranoproliferative Glomerulonephritis ◽

Igg Subclasses

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P56 A rare case of Sjögren's nephropathy

Rheumatology ◽

10.1093/rheumatology/keaa111.055 ◽

2020 ◽

Vol 59 (Supplement_2) ◽

Author(s):

Constanta Amoasii ◽

Rosalind Benson ◽

Janice Harper ◽

Devesh Mewar

Keyword(s):

Lupus Nephritis ◽

Renal Disease ◽

Renal Biopsy ◽

Kidney Injury ◽

Left Knee ◽

Fibrillary Glomerulonephritis ◽

Pet Ct ◽

Ckd Stage ◽

Glomerular Deposits ◽

Anti Phospholipid Syndrome

Abstract Background A presentation of acute kidney injury (AKI) in a patient with a longstanding diagnosis of SLE nephritis was challenged following unexpected renal biopsy and immunology resulting in a diagnosis of primary Sjögren's Syndrome (pSS) related fibrillary glomerulonephritis (FGN). Methods A 62 year old Caucasian female presented with AKI and infected leg ulcer. Previously diagnosed with SLE (1996), anti-phospholipid syndrome and non-biopsy proven lupus nephritis (LN), CKD stage G2/3 she was on Hydroxychloroquine and lifelong anticoagulation. Examination revealed bilateral pitting oedema up to knees and a large non healing skin ulcer to left knee. Investigations showed new anaemia (Hb 75g/dl, MCV 94), lymphopaenia, urea- 25 (9.8), Creatinine- 280 (97), urine protein 6.88g/24 hr. USS KUB, ECHO, CXR, virology screen, PET CT were unremarkable. Immunology studies revealed ANA 1:160, anti-Ro, anti-La, but normal DsDNA and C3, C4. In the light of reported sicca symptoms and serology the diagnosis was revised to pSS. Renal biopsy was initially reported as diffuse lupus nephritis (Class IV) with crescents, and focal weak mesangial IgG and C3 but no glomerular deposits. Electron microscopy (EM) demonstrated abundant mesangial and intramembranous deposits with a fibrillary substructure diagnostic of FGN. Immunosuppression with pulsed intravenous (IV) methylprednisolone and IV Cyclophosphamide were given. She developed acute arterial intercostal bleeding at biopsy site and further significant lower gastrointestinal bleeding unamenable to endoscopic therapy or embolisation and the patient died. Results This case highlights the importance of ensuring that serology is interpreted correctly alongside histopathology and urinalysis in the diagnosis of CTD related renal disease. PSS related nephropathy is seen in less than 10% of patients. Tubulointerstitial nephritis (TIN) accounts for 2/3 of patients and glomerulonephritis (GN), typically membranoproliferative GN (MPGN) for most others. FGN in pSS is very rare, with only 3 reported cases to date. Like lupus nephritis, FGN can present with AKI, hypertension and nephrotic range proteinuria. However, renal biopsy proves invaluable in differentiating between the conditions. In FGN characteristically on biopsy disorganised, non-branching fibrils are seen in all glomerular compartments and are negative for congo red stain further differentiating from amyloid FGN. Immunofluorescence microscopy shows uniform staining of the fibrils for immunoglobulins (mostly subclass IgG4). FGN has a poor prognosis with patients developing severe chronic renal failure despite immunosuppression. Conclusion Considering surprising investigation results, it is crucial we rechallenge the validity of longstanding diagnoses as highlighted by our case. In proven pSS with systemic features we recommend screening all patients for renal disease annually including serum electrolytes, urine dipstick, protein creatinine ratio and pH. If abnormalities are identified, renal US and consideration of biopsy is crucial in establishing aetiology as management and prognosis differs between subtypes. Disclosures C. Amoasii None. R. Benson None. J. Harper None. D. Mewar None.

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