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.

Post Renal Transplant Collapsing Glomerulopathy is Associated with Poor Outcomes.

INTRODUCTION: Collapsing glomerulopathy (CG) is a distinct morphologic pattern of proliferative renal parenchymal injury. It differ from focal segmental glomerulosclerosis (FSGS) by clinicopathologic pattern and its adverse outcome. The clinical significance of CG in renal allograft biopsies is not yet clear due to scant data and less occurrence of CG in renal transplant recipients. We conducted this single-center retrospective study to evaluate the prevalence, clinicopathological features, and outcome of post renal transplant CG. SUBJECTS AND METHODS: We studied 127 renal allograft biopsies performed over a period of 45 months (Jan 2015-Oct 2018). A diagnosis of CG was made if at least one glomerulus demonstrated global or segmental collapse of the glomerular capillary walls, associated marked hyperplasia, and hypertrophy of the overlying visceral epithelial cells. We analyzed clinical, biochemical, and pathological characteristics and its impact on renal allograft outcome. Statistical analysis was performed and continuous variables were expressed as means ± standard deviation (SD) or medians (interquartile range and noncontinuous data were expressed in percentage and numerical values. RESULTS: The prevalence of CG was 5.3% (7/127) of allograft biopsies. Out of the seven patients, six patients had undergone live donor transplant and one patient had undergone deceased donor renal transplant. The native kidney disease was unknown in these patients except one (IgA nephropathy). The median duration of diagnosis for CG was 17 months after transplantation (range 5-132months). Presenting symptoms were pedal edema and hypertension in 71.4% (5) patients each. All patients had proteinuria of more than 1 gm and renal allograft dysfunction and median serum creatinine of 3.05 mg/dl (1.5-4.8 mg/dl). All patients received standard triple immunosuppression. Over a period of 2-20 months, 57.14% (4) patients developed a graft failure and 43% (3) of the other patients had functioning grafts with serum creatinine of 1.5-4.2 mg/dl. CONCLUSIONS: CG presents with moderate to severe proteinuria and may lead to rapid graft dysfunction and subsequent graft failure in most of the patients.

Atheroembolic Renal Disease: A Case Series.

Atheroembolic renal disease (AERD), a part of systemic cholesterol embolization syndrome, is caused by the occlusion of small arteries in the kidneys by cholesterol crystal emboli from ulcerated atherosclerotic plaques. Kidney is commonly involved because of its proximity to the abdominal aorta and its enormous blood supply. AERD is an under diagnosed condition. We report eight cases of AERD, highlighting the variability in its clinical presentation and the importance of a renal biopsy to arrive at a definitive diagnosis.

Dense deposit disease: a greatly increased biopsy incidence in India versus the USA.

BACKGROUND: We present the largest clinicopathologic case series to date of dense deposit disease (DDD) in an Indian population and compare the renal biopsy incidence rate to that seen in a large renal laboratory in USA. METHODS: Cases of DDD were identified and evaluated from native kidney biopsies reported at Renopath, India and at Arkana Laboratories, in the USA. Renopath receives biopsies from four states, located in the South and Eastern part of India. Arkana Laboratories' biopsies came from 37 states across the USA. RESULTS: During the study period, there were a total of 25 patients diagnosed with DDD among the 7335 native kidney biopsies at Renopath. Thus, the biopsy incidence rate (cases of DDD/total renal biopsies/year) is 0.0034. By comparison, there were 10 cases of DDD diagnosed among 26 319 native kidney biopsies at Arkana Laboratories during the same time period, with a renal biopsy incidence rate of 0.00038. CONCLUSIONS: DDD in this Indian subpopulation has similar clinical and pathologic characteristics when compared to previously reported studies. However, the biopsy incidence rate is about 890% or 8.9 times more common in this subset of the Indian population when compared with a broad cross-section of the US population. In addition to potential genetic factors, environmental conditions and chronic infections likely contribute to the markedly higher biopsy incidence rate. Given the much greater number of patients with DDD in this population, further retrospective and prospective studies would allow more rapid progress in understanding the pathogenesis of DDD and thus potential treatment of patients with DDD.