Clinicopathological profile and outcome of adult infection-related glomerulonephritis: A prospective follow-up study.

Background: . Infection-related glomerulonephritis (IRGN) in adults is witnessing a dramatic shift in its epidemiology and outcome. Adult IRGN studies are all retrospective in nature, and Indian studies are scarce. Methods: . We did this prospective study (September 2016-April 2018) on all patients with biopsy-proven IRGN and age ≥18 years satisfying three of five diagnostic criteria. Patients with persistent hypocomplementaemia (>3 months) were excluded. We did electron microscopy in those without a minimum of three diagnostic criteria and did an extensive search for any occult infection in every patient. Results: . Forty-five patients were studied with a mean (SD) follow-up of 45.7 (20) weeks. Their mean age was 41.5 years (18-70 years), with a female preponderance (1:1.25). At presentation, the majority had oedema (100%), oliguria (84.4%), hypertension (80%) and haematuria (77.8%). Of them, 86.7% had renal insufficiency and 35.6% required dialysis. Only 53.3% of them had evidence of antecedent/ current infection, with skin/subcutaneous focus being the most common site. Hypocomplementaemia was present in 82.2% of patients. Salient pathological features were endocapillary proliferation (93.3%), neutrophilic infiltration (88.9%), presence of crescents (17.8%), interstitial infiltration (24.4%), moderate-to-severe interstitial fibrosis with tubular atrophy (IFTA; 15.5%) and underlying diabetic glomerulosclerosis (8.9%). Only 66.7% of patients made complete renal recovery. By logistic regression analysis, the predictors of poor outcome were a requirement for dialysis at presentation (p=0.04) and presence of IFTA (p = 0.03). Conclusion: . A proportion of adult IRGN patients progress to chronic kidney disease.

Systemic Lupus Erythematosus and Myasthenia Gravis: A Rare Association.

Systemic lupus erythematosus (SLE) and myasthenia gravis (MG) are two autoimmune diseases that have a higher incidence in young females, relapsing-remitting course, and positive antinuclear antibodies. SLE and MG are two different clinical syndromes, which can coexist or precede each other; however, their occurrence in the same patient is rare. We report a 38-year-old female with biopsy-proven lupus nephritis on steroids and cyclophosphamide, later developed MG. Nerve conduction studies showed the decremental response of 15%-25% over facial muscles with no decremental response over limb muscles. Although antianticholinesterase receptor (AchR) antibodies were negative, she was treated with oral pyridostigmine 60 mg twice daily and clinical improvement of ocular symptoms was seen within 48 h. At present, she is on oral prednisolone and mycophenolate mofetil with follow-up creatinine of 1.4 mg/dl and no neurological symptoms.

Spectrum of Hypokalemic Paralysis from a Tertiary Care Center in India.

Hypokalemic paralysis is an important and reversible cause of acute flaccid paralysis. The treating physician faces unique diagnostic and therapeutic challenges. We did a prospective study and included all patients with acute flaccid weakness and documented serum potassium of <3.5 mEq/L during the period between January 2009 and August 2015. We studied the incidence, etiology, clinical profile, and acid-base disturbances in patients presenting with hypokalemic paralysis and analyzed the significance of periodic and non-periodic forms of hypokalemic paralysis on patient's outcome. Two hundred and six patients were studied with a mean follow-up of 3.6 ± 1.2 years. Mean age was 37.61 ± 2.2 years (range 18-50 years). Males were predominant (M:F ratio 2.1:1). The nonperiodic form of hypokalemic paralysis was the most common (61%). Eighty-one (39%) patients had metabolic acidosis, 78 (38%) had normal acid-base status, and 47 (23%) patients had metabolic alkalosis. The most common secondary cause was distal renal tubular acidosis (RTA) (n = 75, 36%), followed by Gitelman syndrome (n = 39, 18%), thyrotoxic paralysis (n = 8, 4%), hyperaldosteronism (n = 7, 3%), and proximal RTA (n = 6, 4%). Patients with non-periodic paralysis had more urinary loss (40.1 vs. 12.2 mmol, P = 0.04), more requirement of potassium replacement (120 vs. 48 mmol, P = 0.05), and longer recovery time of weakness (48.1 vs. 16.5 h, P = 0.05) than patients with periodic paralysis. Non-periodic form of hypokalemic paralysis was the most common variant in our study. Patients with periodic paralysis had significant incidence of rebound hyperkalemia.

Collapsing Glomerulopathy and Thrombotic Microangiopathy in Postpartum Period: Two Case Reports.

Collapsing glomerulopathy (CG) is a distinct histopathologic pattern of glomerular injury characterized by global/segmental wrinkling of the glomerular basement membrane with podocyte hyperplasia and hypertrophy along with tubulointerstitial changes. There is no specific treatment for CG due to etiological heterogeneity, and newer insights into the pathogenesis may lead to the development of targeted therapy. The most common form of CG is the primary or idiopathic followed by secondary (due to viral infections, autoimmune disease, drugs, etc.) and genetic causes. Thrombotic microangiopathy (TMA) is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and organ failure of variable severity. We here present two young women with preeclampsia who presented with acute kidney injury, anemia, and schistocytes in peripheral smear suggestive of TMA. Renal biopsy showed interesting histopathology of CG in addition to TMA in the first patient and CG alone in the second. Both the patients received supportive therapy while the first patient also received plasmapheresis. One patient had complete recovery, and other had partial recovery of renal function at last follow-up. Combined histopathological lesion of CG with TMA has never been reported in postpartum period so far in literature.

IgA-dominant Infection-related Glomerulonephritis in India: A Single-center Experience.

IgA-dominant infection-related glomerulonephritis (IRGN) is a distinct morphologic variant of IRGN, characterized by dominant or codominant glomerular deposits of IgA, mostly in elderly and patients with diabetes. More cases are being reported in recent times due to increased awareness of the disease entity and increased rate of Staphylococcus infection. It usually presents as rapidly progressive renal failure with proteinuria, and treatment guidelines for this disease entity are not well defined. We report here 12 cases of IgA-dominant IRGN seen over a period of 5 years from a single center. Clinical features, biopsy findings, treatment, and outcomes were analyzed. Out of 12 patients, eight were males. The mean age of presentation was 52.4 ± 21 years. Skin was the most common site of infection seen in six patients. Gross hematuria was seen in 4 patients and 11 had nephrotic proteinuria. Eleven had low serum C3. Only two patients had diabetes. Methicillin-resistant Staphylococcus aureus (MRSA) was the most common organism isolated in six patients. Most common histopathology was crescentic glomerulonephritis seen in seven patients, followed by endocapillary proliferation in three and diffuse proliferative glomerulonephritis in two. Hemodialysis was done in eight patients and six patients received steroid therapy. End-stage renal disease developed in three patients, chronic kidney disease in three, and three patients died due to sepsis. Various infections including MRSA and Escherichia coli were associated with IgA-dominant IRGN both in patients with diabetes and nondiabetics. Suspicion and recognition of the disease is important as it has therapeutic and prognostic implications.

Scrub typhus meningitis in a renal transplant recipient.

Scrub typhus is a rickettsial infection commonly seen in Asia. The clinical presentation ranges from nonspecific febrile illness to potentially fatal multiorgan involvement such as liver, kidney, or lung. Central nervous system involvement is uncommon. We report a 45-year-old female renal transplant recipient who presented with fever, headache, meningeal signs, graft dysfunction, and eschar. IgM antibodies against Orientia tsutsugamushi were positive by enzyme-linked immunosorbent assay. Despite oral doxycycline therapy for 5 days, she did not improve but responded well to intravenous azithromycin. To the best of our knowledge, scrub typhus as a cause of meningitis in a renal transplant recipient has not been reported so far.

Thrombotic microangiopathy due to Viperidae bite: Two case reports.

Snake bite is mainly an occupational hazard and causes serious health problems in rural India. Acute kidney injury (AKI) occurs in 5-30% cases. Renal pathologic findings include acute tubular necrosis, cortical necrosis, interstitial nephritis, glomerulonephritis, and vasculitis. Thrombotic microangiopathy (TMA) occurrence after a snake bite is reported rarely. Here, we present two patients who developed TMA after viper bite treated with hemodialysis and plasmapheresis. Renal biopsy showed fibrin thrombi in glomeruli and arterioles with cortical necrosis. One patient progressed to end-stage renal disease and other was lost to follow-up. TMA should be considered as a possible pathogenesis of AKI after snake bite. The role of plasma exchanges in snake bite TMA is yet to be defined.

Thrombotic microangiopathy: An unusual cause of renal failure in rheumatoid arthritis.

Rheumatoid arthritis (RA) is one of the commonest rheumatological diseases. Renal involvement is not common but can occur as a result of chronic inflammation as part of disease process or drug toxicity. Thrombotic microangiopathy (TMA) is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and organ failure of variable severity. Only a few cases of TMA in patients with RA were reported to date. We describe a 45-year-old female patient with RA who presented with oliguria and edema. Renal biopsy showed TMA with patchy cortical necrosis. She improved with hemodialysis and plasmapheresis.

Deceased donor renal transplantation: A single center experience.

Deceased donor renal transplantation (DDRT) constitutes less than 5% of all kidney transplantats in India. A retrospective analysis of 173 deceased donor renal transplants performed in a public funded government hospital was done. Mean age of the recipients was 36 years (male:female ratio 2.4:1), and that of the donors was 32.3 years (male:female ratio 6:1). The cold ischemic time was 340 ± 170 minutes. Mean follow-up period was 36 months. Forty one patients died, 75% of them in the first post - transplant year. Sepsis and cardiovascular disease were the most common causes of death. Twenty two percent had acute rejection. There was no significant difference in the incidence in the rate of acute rejection, bacterial, fungal infections and death rate between the cohorts of induction and non induction immunosuppression. The patient and death censored graft survival at 1 year were 80 and 82.6% and at 5 years were 76 and 80% respectively.

Clinicopathological correlation and treatment response of primary focal segmental glomerulosclerosis in adults and adolescents.

The incidence of focal segmental glomerulosclerosis (FSGS) is approximately 10% in children <6 years, 20% in adolescents, and 20-25% in adults. A retrospective observational study was done to document clinicopathological correlation, treatment response, and risk factors in the progression of chronic kidney disease (CKD) of primary FSGS in adults and adolescents. A total of 170 patients were studied with a mean follow-up of 4.32 ± 1.2 years. FSGS not otherwise specified was the most common subtype (56%) followed by tip variant (24%). About 32% had complete remission (CR) at a mean time of 6.4 months, 23% had partial remission (PR) at a mean time of 5.7 months, and 45% had no response to steroids. Persistent nephrotic proteinuria at 3rd and 6th month and presence of interstitial fibrosis and tubular atrophy >30% in renal biopsy are the independent predictors of poor response to treatment. Presence of anemia, interstitial fibrosis, and tubular atrophy of >30% in renal biopsy and the absence of remission after treatment were the independent predictors of CKD progression. Overall renal survival was 78% at 3 years and 54% at 5 years. Renal survival difference with or without nephrotic proteinuria at onset was 39% and 69% at 5 years. Renal survival was higher in patients with normal renal function (66%) compared with those who had renal failure (42%) at 5 years. Renal survival at 5 years for CR was 69%, PR was 49%, and no remission was 42%.

Two consecutive recurrences of crescentic immunoglobulin A nephropathy in a renal transplant recipient.

We report a 21-year-old male who developed end-stage renal disease, probably due to immunoglobulin A nephropathy (IgAN), received a renal transplant from his mother, which was lost due to crescentic IgAN after 18 months. Two years later, he received a second transplant from a deceased donor. He developed rapidly progressive graft dysfunction 3 years later. Allograft biopsy revealed crescentic IgAN, which was successfully treated with intravenous steroids and cyclophosphamide. Recurrence of IgAN in two successive allografts in one patient has not been reported previously.

Familial Lecithin Cholesterol Acyl Transferase Deficiency with Chronic Kidney Disease.

Familial lecithin-cholesterol acyltransferase (LCAT) deficiency is a rare autosomal recessive (AR) disease caused by mutation in the LCAT gene. LCAT enzyme esterifies cholesterol molecules in high-density lipoprotein(HDL) and low density-lipoprotein (LDL) particles. This enzyme deficiency is characterised by progressive corneal opacification, glomerulopathy, mild - moderate haemolytic anaemia and very low plasma levels of HDL. We here report a 34 year-old lady who presented with hypertension, nephrotic proteinuria, renal failure, corneal ring opacities, anemia and dyslipidemia. The diagnosis of familial LCAT deficiency was confirmed by clinical examination, characteristic dyslipidemia, undetectable LCAT levels in plasma and positive family history.

Prevalence of serum anti M-type phospholipase A2 receptor antibody in primary membranous nephropathy: A single center experience.

We conducted a prospective study to assess utility of detection of antibodies to phospholipase A2receptor (PLA2R) in the serum of patients with membranous nephropathy. Seventy five patients with biopsy proven membranous nephropathy admitted between January 2011 and September 2014 were studied. Serum anti- PLA2R was tested by indirect immunofluorescence. The test was positive in 45 out of 60 patients with primary membranous nephropathy (PMN) and in none of the 15 patients with secondary membranous nephropathy, with a sensitivity of 75% and specificity of 100% for PMN. Anti PLA2R positivity also showed a significant correlation with quantum of proteinuria and negative correlation with serum albumin. This study has validated detection of serum anti PLA2R in PMN as a non invasive diagnostic tool in Indian patients.

Acute kidney injury and disseminated intravascular coagulation due to mercuric chloride poisoning.

Mercury is a toxic heavy metal and occurs in organic and inorganic forms. Inorganic mercury includes elemental mercury and mercury salts. Mercury salts are usually white powder or crystals, and widely used in indigenous medicines and folk remedies in Asia. Inorganic mercury poisoning causes acute kidney injury (AKI) and gastrointestinal manifestations and can be life-threatening. We describe a case with unknown substance poisoning who developed AKI and disseminated intravascular coagulation (DIC). Renal biopsy showed acute tubular necrosis. Later, the consumed substance was proven to be mercuric chloride. His renal failure improved over time, and his creatinine normalized after 2 months.

Angiomyolipoma of donor kidney: Successful transplantation and 5-year follow-up.

Angiomyolipomas (AML) of the kidney are non-encapsulated benign neoplasms with the incidence of 45-80% in patients with tuberous sclerosis and 1-3% in sporadic cases. There are very few case reports in the literature in which kidneys with AML have been used for transplantation. We report here a 27-year-old female patient who received a live related renal transplant from her mother with isolated angiomyolipoma in donor kidney and on follow-up after 5 years, has stable graft function and tumor size.

Lymphomatous Infiltration of Kidneys.

Renal involvement in lymphoma usually occurs late in the course of the disease and is clinically silent. Acute kidney injury (AKI) is rare, with the reported incidence of 0.5% in the literature. We describe here a 20 years old female patient who presented with non-dialysis requiring AKI and renal biopsy showed lymphomatous infiltration of kidneys. Though renal function improved, patient died due to central nervous system involvement.

IgG4 related interstitial nephritis: A case report and review of literature.

IgG4 interstitial nephritis is a recently described entity. A middle-aged gentleman with bilateral parotid enlargement, hepatosplenomegaly and generalized lymphadenopathy was referred to us for evaluation of renal failure. He had trace proteinuria and large kidneys. Kidney biopsy revealed interstitial nephritis with characteristic storiform fibrosis. Immunohistochemistry demonstrated intense staining for IgG4-secreting plasma cells in the interstitium.