Complementary and Alternative Medicine Use and Glomerular Disease: A Contemporary Case Series.

Rationale & Objective: Complementary and alternative medicine (CAM) intake is widely prevalent in many parts of India. Heavy metals are known ingredients in some of these formulations. We studied the spectrum of glomerular diseases in patients using CAM. Study Design: Case series. Setting & Participants: Patients with proteinuria or unexplained acute kidney injury, who underwent a kidney biopsy between May 2021 and September 2022, and who provided a history of recent CAM intake were included in the study. For patients enrolled prospectively, blood and urine samples were analyzed using mass spectrometry for the presence of mercury, lead, arsenic and cadmium. The CAM formulation, when available, was analyzed using inductively coupled plasma-optical emission spectroscopy. Results: Twenty-eight patients were enrolled in the study, with a median duration of CAM intake of 4 months (interquartile range, 2-6 months). Heavy metal screening was performed in 17 patients, of whom 15 had elevated urine mercury levels, 10 had elevated blood mercury levels, and 1 had elevated blood and urine arsenic levels. Of the 6 CAM formulations that were analyzed, all had high levels of mercury. Kidney biopsy findings were membranous nephropathy (n = 19), minimal change disease (n = 8), and mesangial proliferative glomerulonephritis (n = 1). Of the 19 patients with membranous nephropathy, 14 were associated with neural epidermal growth factor-like protein 1 (NELL-1). With conservative management alone, 17 patients achieved complete remission. Limitations: Not all patients underwent blood and urine mercury testing, and only 6 patients provided the CAM samples for analysis. Furthermore, occupational and residential exposure to mercury could not be excluded. Conclusions: The most common kidney pathology noted in our study was membranous nephropathy, which was predominantly associated with neural epidermal growth factor-like protein 1. A significant proportion of the patients recovered completely after withdrawal of the offending agent and initiation of renin-angiotensin system blockade.

Complementary and alternative medicine (CAM) intake is widely prevalent in many parts of India, and heavy metals are known ingredients in some of these formulations. We describe the clinical spectrum of kidney disease, among patients who had recently ingested CAM. All patients underwent a kidney biopsy, and the most common finding was an entity called "NELL-1-associated membranous nephropathy," which is known to be associated with heavy metal toxicity and CAM intake. Of 17 patients screened for such heavy metals, 15 had greater-than-permissible levels of mercury. Furthermore, 6 patients provided the CAM formulations that they had consumed for analysis: all contained high levels of mercury. Most such patients recovered following withdrawal of the offending CAM agent.

Abdominal Pain in a Female with Lupus - Opening the Pandora's Box.

Systemic lupus erythematosus (SLE) is an autoimmune disease that can involve multiple organ systems. The most common form of vasculitis seen in SLE is small vessel vasculitis. Aortitis in SLE or antiphospholipid syndrome is an extremely rare complication. Here, we present a 32-year-old female who presented with a history of prolonged abdominal pain, who was evaluated and diagnosed to have aortitis as an unusual involvement in SLE with secondary antiphospholipid antibody syndrome.

Karyomegalic Interstitial Nephritis in the Allograft Kidney - A Case Report.

Karyomegalic interstitial nephritis is a rare progressive renal disease. We report a 36-year-old male patient who developed kidney failure due to this condition, underwent kidney transplantation from his sister, and developed the same condition in the graft. Genetic testing of the donor revealed autosomal recessive compound heterozygous mutation of Fanconi anemia-associated nuclease1 (FAN1) gene. Karyomegalic interstitial nephritis is most probably donor derived in our patient. It should not be mislabeled as viral nephropathy.

IgA Nephropathy - Beyond the Renal Cortex.

Immunoglobulin A (IgA) nephropathy is the most common glomerular disease worldwide. It usually presents as a nephritic syndrome with macroscopic hematuria, oliguria, and proteinuria with or without azotemia. Rapidly progressive glomerulonephritis with crescents is being described in about 30% of cases and is mostly associated with nephrotic-range proteinuria, accelerated hypertension, and accelerated decline toward end-stage renal disease. Medullary angiitis is a rare finding in renal biopsy and is usually associated with pauci-immune glomerulonephritis. We describe a rare association of medullary angiitis in IgA nephropathy, probably the first reported case in the country.

A Case of Posttransplant Fibrillary Glomerulonephritis.

Fibrillary glomerulonephritis (FGN) is a rare form of glomerulonephritis, usually occurring in concurrence with other conditions such as hepatitis C, dysproteinemia, autoimmune conditions, diabetes mellitus, and malignancy. The diagnosis is made by the presence of randomly oriented fibrillar deposits with a mean diameter of 20 nm, which stain positive for IgG and C3 and are negative for congo red and thioflavin T stains. Staining for DNAJB9 (DnaJ homolog subfamily B member 9) is a recently discovered mode of diagnosis of FGN without electron microscopy. The prognosis is poor and optimal treatment is yet not clearly defined, though rituximab may be useful in FGN patients with relatively preserved renal functions. In this case report, we discuss a case of post-renal transplant patient with de novo occurrence of fibrillary glomerulonephritis.

Hemoglobin Casts in Kidney Biopsies: Etiological Spectrum.

Intravascular hemolysis, which is the destruction of red blood cells in circulation, can cause acute kidney injury as the hemoglobin released by the lysed cells is toxic to the tubular epithelial cells. We performed a retrospective analysis of 56 cases of hemoglobin cast nephropathy reported at our institution to analyze the etiological spectrum causing this rare disease. The mean patient age was 41.7 (range: 2-72 years), and the male-to-female ratio was 1.8:1. All patients presented with acute kidney injury. The etiologies include rifampicin-induced, snake bite, autoimmune hemolytic anemia, falciparum malarial infection, leptospiral infection, autoimmune hemolytic anemia, sepsis, non-steroidal anti-inflammatory drugs, ingestion of termite oil, heavy metal poisoning, wasp sting, and valvular heart disease with severe mitral regurgitation. We demonstrate a wide spectrum of conditions associated with hemoglobin casts in the kidney biopsy. Hemoglobin immunostain is required to establish the diagnosis.

Efficacy of Corticosteroids in Infection-Related Glomerulonephritis-A Randomized Controlled Trial.

Introduction: Infection-related glomerulonephritis (IRGN) is associated with glomerular immune complex deposition along with complement activation. Steroids may attenuate glomerular injury and thereby improve renal outcomes. Methods: We randomly assigned patients who had biopsy-proven IRGN and serum creatinine greater than 1.5 mg/dl to receive corticosteroids plus supportive care (intervention arm), or supportive care alone (control arm). Patients were followed up for 6 months. The primary outcome was complete renal recovery at 6 months. Safety of steroid therapy was also assessed. Results: A total of 52 patients underwent randomization. At 6 months, 17 of 26 patients (65.4%) in the intervention arm and 14 of 26 patients (53.8%) in the control arm had complete renal recovery (odds ratio 1.6; 95% confidence interval, 0.5-4.9; P = 0.397). There was no statistically significant difference in any of the secondary outcomes. Adverse events occurred in 12 patients (46.2%) in the intervention arm and 2 patients (7.7%) in the control arm (P = 0.002). Conclusion: In this single-center trial, corticosteroids did not result in a statistically significant increase in rates of complete renal recovery at 6 months. There was a significantly increased risk of adverse events associated with the use of corticosteroids.

Clinical Profile, Histopathology, and Outcomes in Infection-Related Glomerulonephritis - Single-Center Experience.

Background: Infection-related glomerulonephritis (IRGN) is an important source of renal morbidity with adverse outcomes in adults. Data from large centers in India is lacking on this common, yet poorly understood entity. Materials and Methods: We performed a prospective observational study of all patients diagnosed with IRGN at our center over a 3-year period between 2017 and 2019. "Typical IRGN" patients were diagnosed based on clinical and laboratory assessment; others underwent renal biopsy. Renal and patient survival outcomes were assessed in addition to factors that help predict outcomes. Results: One hundred and twenty-five patients with a diagnosis of IRGN were included in the study, including 86 patients who underwent renal biopsy. This represented 24% of all biopsies during this time period, and IRGN was the most common nondiabetic kidney disease identified in diabetic biopsies at our center. Female preponderance and a seasonal variation were striking. Atypical sources of infection like otomycosis, tooth abscess, and dengue virus infection were noted. Male gender and diabetes were important risk factors for severe disease. Rapidly progressive glomerulonephritis (RPGN), atypical serum complement profiles, and comorbid illnesses were common in adults. Though children had more benign disease and outcomes, life-threatening complications were also noted. C3 dominance was the most striking immunofluorescence (IF) finding and was associated with poorer outcomes. Crescentic IRGN was rare, and four cases of IgA-dominant IRGN were noted. Also, 24% of the cohort required renal replacement therapy. RPGN presentation of IRGN portended worst prognosis with end-stage renal disease (ESRD) in 31% and death in 22% of patients. Conclusion: IRGN is a common clinical entity in adults with the potential for adverse renal and survival outcomes. We have identified clinical and biopsy characteristics that are associated with ESRD and death.