Spectrum of renal vascular lesions among patients with lupus nephritis: An experience from a tertiary care center.

Background: Lupus nephritis (LN) is the assemblage of glomerular, tubulointerstitial and vascular changes. Despite the fact that glomerular changes are overemphasized in pathological classification and scoring system, but the existence of vascular damage negatively impact the clinical course. Aims and Objective: This study was conducted to determine the clinicopathological spectrum of renal vascular lesions in lupus nephritis. Materials and Methods: Renal microvascular lesions in biopsy proven lupus nephritis were classified into 5 major categories-thrombotic microangiopathy, true vasculitis; lupus vasculopathy, uncomplicated vascular immune deposits, and arterial. Clinical details, laboratory parameters and histopathological variables were compared among all groups. Summary of chronic changes was also assessed. Results: Biopsies from 56 patients revealed thrombotic microangiopathy (2), lupus vasculopathy (3), uncomplicated vascular immune deposit (6), PAN type vasculitis (1) and arterial sclerosis (13). No renal vascular lesions were found in 35.18% of patients. At the time of biopsy, arterial sclerosis or lupus vasculopathy patients were older Nephritis subtype. Activity indices were higher in lupus vasculopathy group whereas patients with arteriosclerosis showed highest chronicity index. Conclusions: Renal vascular lesions are common in systemic lupus erythematosus patients with nephritis and may be associated with aggressive clinical course.

Heavy chain deposition disease in a case of clear cell renal cell carcinoma- A jack in the box.

Renal cell carcinoma (RCC) is the most common subtype of adult renal tumors, and its detection rate in the early stages has been increased in the dawn of advanced imaging modalities. Nephrectomy is the mainstay of treatment; determination of tumor category and staging is the primary concern of oncopathologists. Non-neoplastic renal parenchyma is overlooked majority of times and thus misses the opportunity to detect concomitant medical renal diseases which also predict the renal outcome in the postoperative era. Although any kind of glomerular or extraglomerular pathology may be encountered, vascular changes in the form of arterionephrosclerosis are the commonest one. Here, we take the opportunity to report an unusual association of heavy chain deposition disease (HCDD) with clear cell subtypes of renal cell carcinoma in a 48-year-old male of Indian ethnicity.

Role of light and immunofluorescence microscopy to differentiate primary and secondary membranous nephropathy.

Context: Membranous nephropathy (MN) causes nephrotic syndrome, mostly primary but may be associated with SLE, infections, cancer, or drug. Aims: To estimate clinical, serological, light microscopic, and direct immunofluorescence (DIF) findings to differentiate primary and secondary MN. Settings and Design: Prospective, cross-sectional, single-center study in a tertiary care hospital. Methods and Material: Total 51 cases from September 2019 to February 2020. Laboratory Data: Blood glucose, urine analysis, urea, creatinine, albumin, cholesterol, HBsAg, Anti HCV, ASO, ANA, MPO ANCA, PR3 ANCA, dsDNA, PLA2R, C3, and C4. Clinical parameters: age, sex, BP, skin lesions, arthralgia, edema, obesity. Renal biopsies examined with H and E, PAS, silver methanamine, MT stains. DIF done with IgG, IgM, IgA, C3c, C1q, kappa, and lambda. Statistical Analysis Used: Statistical software (Graph Pad PRISM 6) and Chi-square test). Results: Among 51 cases, 25 are primary and 26 are secondary MN with 22 being lupus nephritis, with 2 being post-infectious and the remaining 2 being proliferative glomerulonephritis with monoclonal immunoglobulin deposition (PGNMIDD) with kappa chain restriction. Mean age was 37 ± 12.18 and 30.69 ± 13.92 years for primary and secondary MN, respectively. Significant male preponderance in primary MN. Serum C4 significantly low in secondary MN (15.34 ± 9.59). Microscopic hematuria present in secondary MN. Mesangial and endocapillary hypercellularity are significant in secondary MN. IgG and kappa are significantly intense in primary whereas IgA, C3c, and C1q are significantly intense in secondary MN. Conclusions: Reliable differentiation between primary and secondary MN has important therapeutic implications.

IgG4 tubulointerstitial nephritis - An uncommon enemy!

IgG4-related disease (IgG4-RD) is an evolving entity characterized by immune mediated multisystem involvement in the form of fibro inflammatory lesions like sclerosing pancreatitis, dacryoadenitis, Reidel thyroiditis, or chronic sclerosing sialadenitis. Barely, the lesions are restricted to kidney (IgG4-RKD: IgG4-related kidney disease) involving either glomerular or extraglomerular compartment. It is challenging to identify and demands an awareness regarding the entity to reduce the number misdiagnosis and missed diagnosis. Here, we report a case of a 45-year-old woman with IgG4 tubulointerstitial nephritis (IgG4-TIN) who presented with unexplained renal dysfunction as her initial manifestation. This is the first case of IgG4-RKD reported from our tertiary care center among 1864 native renal biopsy in the last two years.

Pediatric lupus nephritis - An evil cousin of its adult counterpart: A single-center based experience from a tertiary care hospital of Eastern India.

CONTEXT: Systemic lupus erythematosus is an autoimmune multisystem disease with a high predilection for renal involvement. Lupus nephritis develops in 20% to 75% within the first two years. Presentation varies from subnephrotic proteinuria to end-stage renal disease. AIMS: To study clinical features, biochemical, and serological parameters and correlate with histological activity and chronicity score [modified National Institute of Health (NIH) score]. SETTINGS AND DESIGN: Retrospective, cross-sectional, single-center based study in a tertiary care hospital of Eastern India. SUBJECTS AND METHODS: We incuded 36 children with lupus nephritis diagnosed from February 2018 to March 2019. Laboratory data included were complete blood count (CBC), blood glucose, urine analysis, serum urea, creatinine, blood urea nitrogen (BUN), albumin, cholesterol, HBsAg, antihepatitis C virus (HCV) antibody, antistreptolysin O (ASO) titer, antinuclear antibody (ANA), myeloperoxidase antineutrophil cytoplasmic antibody (MPO ANCA), proteinase 3 antineutrophil cytoplasmic antibody (PR3 ANCA), double-stranded DNA (dsDNA), C3, and C4. Clinical parameters were age, sex, blood pressure (BP), skin lesions, arthralgia, edema, obesity. Renal biopsies examined with light microscopy, hematoxylin and eosin (H and E), periodic acid-Schiff (PAS), silver methanamine, Masson's trichrome (MT) stains. Immunofluorescence microscopy done with IgG, IgM, IgA, C3c, C1q, kappa, lambda antibodies. STATISTICAL ANALYSIS USED: Kruskal-Wallis and χ2 tests. RESULTS: Mean age was 15.12 ± 3.49 and 12.5 ± 1.73 years for lupus nephritis (LN) with activity and LN without activity, respectively. Mean dsDNA was higher and mean C3 was lower (52.35 ± 22.21 mg/dl) in active LN. Mean 24-hour urinary protein was higher in LN without activity. Serum creatinine was raised in active LN. LN class III and IV showed higher activity than chronicity. CONCLUSIONS: Pediatric LN is proliferative and more active as compared with adult counterparts. Activity scores are much higher than chronicity scores.

Granulomatous Interstitial Nephritis - A Series of Six Cases.

Granulomatous interstitial nephritis is an uncommon variant accounting for about 6% of all tubulointerstitial nephritis. The etiology can be drugs such as antibiotics and nonsteroidal anti-inflammatory drugs and infections such as tuberculosis, sarcoidosis, and fungal infections. Renal biopsy remains the gold standard for establishing the diagnosis. Here, we present a series of six cases of granulomatous interstitial nephritis, of which two cases were associated with lupus nephritis and another two cases with crescentic glomerulonephritis. Focal segmental glomerulosclerosis and mesangiosclerosis with chronic tubulointerstitial nephritis were detected in the rest of the cases. Most of the patients presented with features of nephrotic syndrome. Urine analysis showed albuminuria in all cases. In renal biopsy, interstitial epithelioid cell granuloma was a constant feature along with which there were foci of necrosis and moderate fibrosis in few cases. But none of our cases had any relevant history of prolonged drug intake. Tuberculosis and fungal infections were also ruled out. Thereby in this case series, we subgroup all the cases into two category four cases associated with granulomatous nephritis and two cases with idiopathic granulomatous nephritis.

Protocol renal biopsy in patients with lupus nephritis: a single center experience.

Renal biopsy plays an indispensable role in the diagnosis and management of patients with lupus nephritis (LN). A number of studies have evaluated the role of a repeat biopsy in case of disease relapse or treatment unresponsiveness. We studied 40 patients with LN with renal biopsies performed at baseline and after six months of therapy. The baseline and protocol biopsies were compared with respect to histological class transformation, crescents, tubular atrophy, interstitial fibrosis and glomerulosclerosis. We also compared serum creatinine, hemoglobin, systemic lupus erythematosus disease activity index (SLEDAI) scores, 24-h urine protein excretion and C3levels as well as activity index (AI) and chronicity index (CI) at baseline and at six months. Comparison of means was made by paired t test, McNemar test and marginal homogeneity test (multinomial data). Histological class transformation was seen in 10 patients (25%). Intra-class progression to greater chronicity was seen in 10 other patients (25%).There was an increase in glomerulosclerosis, tubular atrophy, interstitial fibrosis and a reduction in cellularity, crescent formation and wire loop lesions in the protocol biopsy. A decline in AI (6.05 vs. 2.50, P <0.001) and SLEDAI scores (8.1 vs. 3.7, P <0.001) and an increase in CI (0.68 vs. 2.52, P <0.001) was observed at the time of protocol biopsy. Our study shows a trend toward greater chronicity in protocol biopsies in LN.

Pulmonary renal syndrome in a case of Wegener's granulomatosis.

We report a case of a 42-year-old patient who presented with Wegener's granulomatosis complicated by pulmonary renal syndrome, i.e., diffuse alveolar haemorrhage and rapidly progressive crescentic glomerulonephritis. The patient was treated with plasmapheresis and immunosuppressive drugs--intravenous cyclophosphamide and methyl prednisolone. The clinical, haematological and biochemical parameters improved substantially and remission is achieved.

Herpes labialis in patients with Russell's viper bite and acute kidney injury: a single center experience.

Snake bite is an important health hazard in tropical countries and is associated with significant morbidity and mortality. Herpes labialis is a common ailment caused by the Herpes simplex virus. There is no published data showing any association between the snake bite and development of Herpes labialis. Here, we present a series of patients who developed Herpes labialis after Russell's viper bite and had acute kidney injury. We attempted to find whether snake bite is an immunosuppressed state and whether it could have pre-disposed the patients to the development of these lesions.