Temporal Arteritis and Vision Loss in Microscopic Polyangiitis: A Case Report and Literature ReviewRead the full article
Case Reports in Nephrology publishes case reports and case series focusing on the prevention, diagnosis, and management of kidney diseases and associated disorders, including cancer. The journal also focuses on advances in transplantation techniques.
Case Reports in Nephrology maintains an Editorial Board of practicing researchers from around the world, to ensure manuscripts are handled by editors who are experts in the field of study.
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Fibrillary Glomerulopathy with a High Level of Myeloperoxidase-ANCA: A Case Report
An elderly woman was admitted with the chief complaint of gross hematuria. Laboratory values indicated a high myeloperoxidase-ANCA level. In renal histological examination, 40% of the glomeruli showed crescent formation, but immunofluorescence staining showed positivity for IgG, C3, and C1q. Furthermore, the deposition of fibrils in the glomerulus was noted on electron microscopy, and immunohistochemical staining showed strong positivity for DNA-J heat shock protein family member B9 (DNAJB9). Crescent formation is a common feature of fibrillary glomerulonephritis (FGN). Thus, in ANCA-positive crescentic glomerulonephritis, immunohistochemical assessments for immunoglobulins and DNAJB9, as well as electron microscopy, are important to correctly diagnose FGN.
Osmotic Demyelination Syndrome in a Patient with Hypokalemia but No Hyponatremia
Osmotic demyelination syndrome (ODS) is characterized by loss of myelin in various parts of the central nervous system. It is mainly caused by a rapid correction of hyponatremia, although other factors that may cause rapid rise in serum osmolality can also be associated with its development. Its prognosis is poor and the recovery rate is unknown. The authors report a rare case of a patient with multiple risk factors for ODS, without hyponatremia, who developed ODS and surprisingly recovered. This case report highlights the importance of recognizing risk factors for the development of ODS, even if the main one is not present.
Liberation from Dialysis Dependence in a Patient with HIV-Associated Nephropathy (HIVAN) after Combined Antiretroviral Therapy (cART)
Prior to the advent of combined antiretroviral therapy (cART), human immunodeficiency virus-associated nephropathy (HIVAN) was inevitably associated with rapidly progressive renal failure and dialysis dependence. HIV-1 seropositive patients often met with untimely deaths due to complications of end-stage renal disease (ESRD), opportunistic infections, or other HIV-related end-organ failure. Although the association between cART and improved outcomes in HIVAN has been recognized for over 20 years, no randomized trials have specifically examined this effect to date. In terms of reversal of dialysis-dependent renal failure after cART initiation, only a handful of case reports exist. The authors report a case of a 44-year-old Latino male requiring thrice-weekly haemodialysis in the setting of biopsy-proven HIVAN who was able to stop dialysis in 7 months after being initiated on cART.
Anterior Ischemic Optic Neuropathy in a Child Receiving Chronic Hemodialysis
Anterior ischemic optic neuropathy (AION) occurs due to hypoperfusion of the optic nerve and is a rare complication in patients receiving maintenance dialysis. To date, AION has only been reported in 22 children, all of whom were receiving peritoneal dialysis. We report the first case of AION in a 2-year 11-month-old child receiving chronic hemodialysis secondary to polycystic kidney disease from a phosphomannomutase 2 gene mutation. This case highlights the consideration for frequent blood pressure monitoring and ophthalmic screening in a certain cohort of children receiving chronic dialysis.
Membranoproliferative Glomerulonephritis Associated with Nivolumab Therapy
Nivolumab is an immune checkpoint inhibitor that targets programmed death-1 on T cells and is designed to amplify an immunologic reaction against cancer cells. However, upregulation of the immune system with checkpoint inhibition is nonspecific, and it can be associated with certain renal side effects, the best documented of which is acute tubulointerstitial nephritis. We present a unique case of a patient with acute kidney injury associated with nephrotic syndrome shortly after starting nivolumab therapy for metastatic anal carcinoma. Subsequent renal biopsy revealed membranoproliferative glomerulonephritis (MPGN). We believe this represents the first reported direct case of nivolumab-associated MPGN. As immunotherapy becomes more widely used in cancer treatment, particular attention must be paid to possible consequences of immune checkpoint inhibitors.
The Various Forms of Nephrotic Syndrome in a Patient with Systemic Lupus Erythematosus
Kidney involvement is frequent in patients with systemic lupus erythematosus (SLE), although it may not be present from disease onset. Renal lupus itself is highly heterogenous with respect to the combination and/or severity of clinical and/or laboratory manifestations. This is a case of a 45-year-old Caucasian female with an established diagnosis of SLE, who presented four times with new onset of proteinuria during a follow-up time of ten years, since the diagnosis of SLE. Specifically, she experienced two episodes of lupus membranous nephropathy, and after she achieved remission, she developed twice overt nephrotic syndrome associated with new and biopsy proven lupus podocytopathy. All these episodes of nephrotic syndrome were combined with systemic symptoms, attributed to lupus itself, while serological activity of lupus was also noted. This case highlights the importance of performing a kidney biopsy in all patients with SLE who have new renal manifestations, including nephrotic proteinuria.