Dense Deposit Disease Involving C3 and C4d Deposits.

Dense deposit disease (DDD), earlier called Type II membranoproliferative glomerulonephritis is distinct disease having frequent relapses reaching end-stage kidney disease by 10-year in up to 50%-60% of cases and high recurrence rate in the allograft. The term DDD is derived from its distinctive ribbon-like osmiophilic deposits in the lamina densa of glomerular basement membrane by electron microscopy. Pathogenetically, alternate pathway dysfunction leads to this disease, which is diagnosed by ultrastructure. Herein, we describe our observation of C4d positivity in an adolescent boy with DDD.

Circulating Monoclonal IgG1-kappa Antibodies Causing Anti-glomerular Basement Membrane Nephritis.

Anti-glomerular basement membrane (GBM) antibody disease is a rare but well-characterized cause of glomerulonephritis. Patients present with rapidly progressive renal failure with hemoptysis. Early diagnosis is crucial in salvaging the renal damage and life-threatening pulmonary hemorrhage. Plasmapheresis and immunosuppression is the mode of therapy. Anti-GBM antibodies are polyclonal in nature. However, rare monoclonal antibodies can cause similar destruction of glomerular capillary walls. We describe distinct combination of circulating monoclonal and anti-GBM nephritis.

Eosinophilic granulomatosis with polyangiitis with severe pulmonary hemorrhage treated with rituximab.

Eosinophilic granulomatosis with polyangiitis (EGPA) is characterized by systemic vasculitis, asthma and eosinophilia. Severe pulmonary hemorrhage is rare. Renal involvement is seen in approximately 25% and can vary from isolated urinary abnormality to rapidly progressive glomerulonephritis. There is limited evidence to support the use of rituximab in this condition. We present a patient with EGPA who had severe pulmonary hemorrhage and rapidly progressive glomerulonephritis. He responded to standard treatment including prednisolone, cyclophosphamide, and plasma exchange. He subsequently had a relapse of pulmonary hemorrhage that was treated successfully with rituximab.

Spectrum of renal involvement in hematolymphoid neoplasms: Renal biopsy findings of 12 cases.

Spectrum of causes for renal dysfunction in patients with hematolymphoid malignancy (excluding plasma cell dyscrasia) is varied. A retrospective evaluation of "native" renal biopsies referred to our institute during the period from January 2010 to December 2013 revealed 12 cases. Age ranged between 7 and 69 (median 54.5) years. All patients were males. The neoplasms included non-Hodgkin lymphoma, chronic lymphocytic leukemia, acute lymphoblastic leukemia, Burkitt's lymphoma, intravascular lymphoma, Hodgkin lymphoma and chronic myeloid leukemia. Proteinuria was noted in 66% of the patients (nephrotic range in 5, subnephrotic range in 3). Renal insufficiency was noted in 100% patients. Malignancy-related kidney injury was noted in 75% of the cases. Renal histology showed lymphomatous infiltration (8), membranoproliferative glomerulonephritis (MPGN) (3), intracapillary monoclonal deposit disease (1) and intravascular lymphoma (1). Distribution of lymphomatous infiltration was diffuse in 50% and focal in 50%. We observed that renal dysfunction was predominantly a direct effect, that is, lymphomatous invasion. Paraneoplastic glomerulopathic changes occur in the form of MPGN. Proteinuria of >2 g/day correlated with glomerular disease.

Atypical presentation of post infectious glomerulonephritis as malignant hypertension and thrombotic microangiopathy.

Infection-related glomerulonephritis presents commonly as acute nephritic illness, hypertension, hypocomplementinemia following an episode of pharyngitis or pyoderma. Atypical features like thrombotic microangiopathy (TMA), produced by neuraminidase antigen targeting endothelium have been described rarely. We report a case of TMA secondary to malignant hypertension, coexisting with post infectious glomerulonephritis.

IgG4 related renal disease: A wolf in sheep's clothing.

IgG4 related disease is a fibro-inflammatory condition with involvement of renal and extra renal organs, characterized by lymphoplasmacytic infiltration with organ dysfunction. We describe three cases of IgG4 related renal disease from a tertiary care hospital in south India.

Sunitinib induced nephrotic syndrome and thrombotic microangiopathy.

Sunitinib is an oral, multitargeted receptor tyrosine kinase inhibitor of targets such as vascular endothelial growth factor and platelet derived growth factor receptor. It is used for the treatment of metastatic renal cell carcinoma (RCC). Use of sunitinib has been associated with renal dysfunction and nephrotic syndrome. However, simultaneous occurrence of nephrotic syndrome and renal dysfunction in a patient treated with sunitinib is rare. We report a case of metastatic RCC treated with sunitinib for 22 months who presented with nephrotic syndrome and renal dysfunction. Renal biopsy was diagnostic of thrombotic microangiopathy with diffuse effacement of podocytic foot process.

Rapidly progressive glomerulonephritis in a patient with renal amyloidosis: Case report and review of the literature.

Renal amyloidosis is characterized by progressive deposition of extracellular material, most commonly in the glomeruli. Most often, patients present with nephrotic range proteinuria and the disease progresses gradually to renal failure. Rapid worsening of renal functions is rare. We report a case of crescentic glomerulonephritis superimposed on amyloidosis, clinically presenting as rapidly progressive renal failure, and present a review of the literature.

Postrenal transplant laryngeal and visceral leishmaniasis - A case report and review of the literature.

Visceral leishmaniasis (kala-azar) is a disease caused by protozoa of genus Leishmania. It is currently regarded as the second most dreaded parasitic disease, next to malaria. There have been very few case reports of visceral leishmaniasis among the renal transplant recipients. We present a renal allograft recipient with symptoms of fever, sore throat, hoarseness of voice, lymphadenopathy, splenomegaly, and pancytopenia after 7 years post-transplant period. On investigating, he was diagnosed to have extensive visceral leishmaniasis with laryngeal involvement. Despite extensive PubMed literature search, we could not find any case report of postrenal transplant visceral and laryngeal leishmaniasis and to the best of our knowledge this is the first case report of this kind.

Diffuse mesangial sclerosis - Report of two cases.

Diffuse mesangial sclerosis (DMS) is a rare cause of nephrotic syndrome in the infantile and childhood period. DMS is a phenotypic expression of syndromic entities such as WAGR syndrome (Wilms' tumor, aniridia, genitourinary anomalies and mental retardation), Denys Drash syndrome, Pierson syndrome, Frasier syndrome, or Galloway-Mowat syndrome. We report two cases of DMS, one presenting in first year of life and another in second decade of life. Both of them had fatal outcome. Recognition of the disease is very important in modifying the management of patient and active surveillance of family members.