IgG4-Related Kidney Disease: A Diagnostic Conundrum Successfully Treated With Steroids and Rituximab.

IgG4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory condition that has been recognized as a unified systemic disease that links many individual organ conditions that were previously considered to be unrelated. The pathological hallmark of the disease is an abundant IgG4-positive plasma cell infiltration in the affected tissues and fibrosis. It is a great mimicker of neoplastic, inflammatory, and infectious conditions. We report a 72-year-old man who presented to our hospital with dyspnea and oliguria. Detailed evaluation revealed that he was treated at multiple places for right-sided loin pain over the past 10 months and was found to have right-sided hydronephrosis, renal dysfunction, and multiple enlarged lymph nodes. A search for underlying malignancy previously was unyielding and he had rapid worsening of renal function prior to the current presentation. He was uremic and was initiated on hemodialysis. Kidney biopsy revealed features of IgG4-related tubulointerstitial nephritis. Despite tubular atrophy and interstitial fibrosis involving more than 50% of the sampled cortex, he showed a good response to steroids and rituximab (RTX) and became dialysis-independent. This report underscores the masquerading presentation of IgG4-RD which can hinder timely diagnosis and demonstrates the usefulness of a regimen of steroids and RTX in its treatment.

Acute Myocardial Infarction in a Patient With Focal Segmental Glomerulosclerosis Tip Lesion: Hit With a Double Whammy.

Nephrotic syndrome is associated with venous and arterial thrombotic complications and is related to the imbalance between pro-thrombotic and anti-thrombotic factors. With an underlying nephrotic syndrome, arterial thromboses are infrequent, and coronary artery thromboses are much rarer. We present the case of a young male, with nephrotic syndrome, who suffered an acute anterior wall ST-segment elevation myocardial infarction. He was subsequently diagnosed to have focal segmental glomerulosclerosis (FSGS)-tip lesion. The patient was successfully managed with thrombolysis, steroids, anticoagulation, antiplatelets, and statins.

Role of Urinary Beta 2 Microglobulin and Kidney Injury Molecule-1 in Predicting Kidney Function at One Year Following Acute Kidney Injury.

BACKGROUND: There is only limited information on the utility of urinary biomarkers in predicting long-term kidney function following acute kidney injury (AKI). The current study assessed whether urinary beta 2 microglobulin/creatinine (B2M/creat) and kidney injury molecule-1/creatinine (KIM-1/creat) ratios, measured in the early recovery phase of AKI, are predictive of kidney function at one year. METHODS: This is a prospective study done in a tertiary care centre in South India, from March 2017 to December 2018. Adult patients who survived an episode of AKI were followed up for one year (n=125). B2M/creat and KIM-1/creat ratio were measured at two weeks and three months following AKI. RESULTS: In the AKI survivors, the B2M/creat ratio at 2 weeks [18.3mg/g (IQR 2.3, 52.9)] and KIM-1/creat ratio [1.1 µg/g (IQR 0.5, 4.0) at two weeks were higher compared to healthy controls [B2M/creat ratio 0.35 mg/g (0.17,0.58) and KIM-1/creat ratio 0.40 µg/g (0.23,1.00); P=<0.001]. After adjusting for covariates, the eGFR and urinary B2M/creat ratio at two weeks following AKI were predictive of eGFR at one year (P<0.001). KIM-1/ creat ratios were not predictive of eGFR at one year. A urinary B2M/creat ratio of 10.85 at two weeks following AKI had an 85.5% sensitivity (95% CI 74, 93) and 64.3% (95% CI 53, 75) specificity to predict CKD at one year. An eGFR cutoff of 60 mL/min/1.73 m2 at two weeks had a sensitivity of 81.8% (95% CI 69, 90) and specificity of 71.4% (95% CI 60, 81) for predicting CKD. The presence of either one criteria (urinary B2M/creat ratio >10.85 (mg/g) or eGFR <60 mL at two weeks) had a sensitivity of 100% (95% CI 94%, 100%) in predicting CKD at one year. CONCLUSION: An eGFR <60 mL/min/1.73m2 and elevated urinary B2M/creat ratio at two weeks following AKI is predictive of low eGFR at one year. Urinary KIM-1/creat ratios do not predict CKD progression.

Idiopathic membranous nephropathy in patients with diabetes mellitus: a diagnostic and therapeutic quandary!

BACKGROUND: Proteinuria and renal dysfunction is common in diabetic patients and may occur due to variety of causes. Nondiabetic renal diseases (NDRD) account for 30% of the renal biopsies, and idiopathic membranous nephropathy (iMN) is a common non diabetic glomerular disease that can exist alone or in combination with diabetic nephropathy (DN). Immunosuppressants used in iMN may be associated with complications of worsening glycemic control and recurrent infections. There is a paucity of literature on the clinical course, outcomes and treatment adverse effects of patients with iMN and diabetes. METHODS: We retrospectively analyzed the data of all diabetics, evaluated for NDRD and found to have iMN, between January 2000 and June 2015 in our institute. RESULTS: A total of 134 patients with diabetes were biopsied for NDRD and 16 patients had iMN. Mean ± standard deviation age was 54 ± 11.77 years and the median duration of diabetes was 9.4 years. Twelve patients had isolated iMN and four patients had iMN coexisting with DN. Response rates of 18%, 35.71% and 63.63% were seen with Modified Ponticelli (MP) regimen, tacrolimus and mycophenolate mofetil (MMF), respectively. Five patients developed treatment-related adverse effects significant enough to necessitate a treatment change. Worsening glycemic control was the most common side effect. Adverse effects were less with the MMF compared with the MP regimen and tacrolimus. CONCLUSION: Patients with iMN coexisting with diabetes exhibit a poor response to the MP regimen. Treatment-related toxicity is less common with MMF in comparison with the MP regimen and tacrolimus-based regimen. An almost similar response was noted with MMF and tacrolimus-based regimen but there was more withdrawal from treatment due to toxicities observed in the latter.