Proteinuria in children with juvenile idiopathic arthritis: Making the case for early urinary screening

Systemic onset juvenile idiopathic arthritis (SOJIA) can be associated with proteinuria due to various renal pathologies. We report two pediatric cases with SOJIA and nephrotic syndrome secondary to renal amyloidosis, a very rare complication in children. Once present, amyloidosis heralds a poor prognosis for the patient, though early detection may allow some improvement if the inflammatory arthritis is controlled

Renal cell carcinoma with t(6,11): A case report and review of literature

Renal cell carcinomas (RCCs) with t(6,11) are very rare tumours. Only a few cases have been reported so far. t(6,11) results in fusion of alpha gene and transcription factor EB (TFEB) gene resulting in the overexpression of TFEB. The specific light and immunohistochemical features help in the diagnosis of this rare type of tumor. We report a case of t(6,11) RCC in a 38-year-old female who was incidentally found to have a right renal mass. We present this case to emphasize the typical light microscopic picture of this extremely rare tumor. Two population of cells are seen: larger cells with abundant cytoplasm and smaller cells with scant cytoplasm. Smaller cells are arranged around hyaline nodules resulting in the formation of characteristic pseudorosettes. Immunohistochemically, these tumors are diffusely positive for vimentin and focally positive for HMB 45 and CD 117. Knowledge about the typical biphasic light microscopic appearance and the characteristic immunohistochemical features help in the diagnosis of this rare type of translocation associated RCC

An overlap of granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis

We present a case report of overlap of granulomatosis with polyangiitis (GPA; formerly known as Wegener’s granulomatosis) and eosinophilic granulomatosis with polyangiitis (EGPA; formerly known as Churg-Strauss syndrome). We report a 45-year-old female who presented with rapidly progressive renal failure associated with fever, polyarthralgia, and respiratory symptoms with cytoplasmic antineutrophilic cytoplasmic antibody (ANCA) and proteinase (PR-3) antigen positivity. Computerized tomography scan of the chest showed diffuse alveolar hemorrhage with renal biopsy revealing pauci-immune necrotizing crescentic glomerulonephritis with intense eosinophilic infiltration suggestive of eosinophilic GPA (EGPA). Our patient had ANCA-associated vasculitis (AAV) with features suggestive of both GPA and EGPA. She was treated with methylprednisolone and cyclophosphamide and attained remission after 2 weeks of therapy. This is a rare report of a patient with AAV having features of both EGPA and GPA

Occurrence of double primary malignancies in an African renal transplant recipient

A 63-year-old African male with end stage renal disease who received a renal transplantation from his daughter after successful treatment of hepatitis C virus, type 1 genotype developed metastatic Kaposi′s sarcoma and subsequently adenocarcinoma of the prostate. He was successfully treated with chemotherapy and reduction of immunosuppression and switch over to rapamycin

Infection-related glomerulonephritis in a renal allograft

Infection-related glomerulonephritis (IRGN) is an immune-mediated glomerulo-nephritis, most commonly caused by bacterial infections. Although there is an increased incidence of infectious episodes in renal transplant recipients, IRGN as a cause of de novo glomerulonephritis is rarely seen probably due to impaired immunity. We hereby report a 28-year-old male renal transplant recipient, who developed IRGN following impetigenous skin lesions after six years of transplant. He developed rapid worsening of allograft function and was started on hemodialysis. Allograft renal biopsy showed diffuse exudative endocapillary proliferation with crescents. Electron microscopy revealed large subepithelial hump-like deposits. Despite pulse steroid therapy, he became dialysis dependent. Our patient is unique in the way that poststreptococcal glomerulonephritis in an adult after renal transplantation has not been reported so far. We conclude that IRGN after renal transplant, though rare is a possible etiology for allograft dysfunction. There is no definitive treatment protocol for this de novo glomerulonephritis which has an overall poor prognosis

Isolated renal involvement of cytomegalovirus inclusion disease in an infant

Cytomegalovirus (CMV) is the most common cause of congenital infections in humans occurring in 1% of all liveborns. Symptomatic congenital CMV infection involves multiple systems and causes significant morbidity and mortality in newborns. Isolated CMV infection of the kidneys in a living infant has not been reported in literature. Here, we report an infant who presented only with renomegaly and renal biopsy showed extensive CMV inclusions. Serum and urine polymerase chain reaction for CMV were positive, and CMV involvement of other organs was ruled out. The child for treated with intravenous ganciclovir and is currently on follow-up. Cytomegalic inclusion disease involving only kidneys without other systems involvement can occur. The treatment protocol is unclear and long-term follow-up is needed

Lupus nephritis in a patient with retroviral infection

A 38-year-old woman, diagnosed as Person Living with Human Immunodeficiency Virus (HIV) on Highly Active Antiretroviral Therapy (HAART) for three years, presented with features of fever, rashes, joint pain, dyspnea and pedal edema. On evaluation, a diagnosis of Systemic Lupus Erythematosus with Lupus Nephritis (LN) triggered by HIV infection was made based on clinical and serological evidence. She was continued on HAART, and immuno-suppressive therapy was co-administered resulting in the resolution of her symptoms. Lupus-like histopathological findings have been reported in patients with HIV-related kidney diseases. This case report is to highlight the co-existence of LN in a patient with HIV infection

COVID-19 Vaccination and New Onset Glomerular Disease: Results from the IRocGN2 International Registry.

KEY POINTS: IgAN and MCD are the most common de novo glomerular diseases reported after COVID-19 vaccination, particularly after mRNA vaccination. Membranous nephropathy, pauci-immune GN, and collapsing GN have also been attributed to COVID-19 vaccination, some with dual histologies. Recovery of kidney function and proteinuria remission is more likely in IgAN and MCD by 4–6 months compared with the other glomerular diseases. BACKGROUND: Patients with de novo glomerular disease (GD) with various renal histologies have been reported after vaccination against SARS-CoV-2. Causality has not been established, and the long-term outcomes are not known. To better characterize the GDs and clinical courses/outcomes, we created the International Registry of COVID-19 vaccination and Glomerulonephritis to study in aggregate patients with de novo GN suspected after COVID-19 vaccine exposure. METHODS: A REDCap survey was used for anonymized data collection. Detailed information on vaccination type and timing and GD histology were recorded in the registry. We collected serial information on laboratory values (before and after vaccination and during follow-up), treatments, and kidney-related outcomes. RESULTS: Ninety-eight patients with GD were entered into the registry over 11 months from 44 centers throughout the world. Median follow-up was 89 days after diagnosis. IgA nephropathy (IgAN) and minimal change disease (MCD) were the most common kidney diseases reported. Recovery of kidney function and remission of proteinuria were more likely in IgAN and MCD at 4–6 months than with pauci-immune GN/vasculitis and membranous nephropathy. CONCLUSIONS: The development of GD after vaccination against SARS-CoV-2 may be a very rare adverse event. Temporal association is present for IgAN and MCD, but causality is not firmly established. Kidney outcomes for IgAN and MCD are favorable. No changes in vaccination risk-benefit assessment are recommended based on these findings