Nephrotic syndrome associated with Tsutsugamushi disease has not been previously reported. We are describing a case of Tsutsugamuchi disease presenting with nephrotic syndrome. A 72-year-old woman presented with fever and generalized edema. Laboratory studies revealed a leukocytosis, hypoalbuminemia, and hypercholesterolemia. Her urine protein excretion was 5.4 g/day. The anti-Tsutsugamushi antibody test was strongly positive (1:2,560). A renal biopsy was performed, and pathologic findings revealed membranous glomerulonephritis. The patient's clinical symptoms improved markedly after treatment with doxycycline.
Aged ; Anti-Bacterial Agents/therapeutic use ; Antibodies, Bacterial/blood ; Biopsy ; Doxycycline/therapeutic use ; Female ; Glomerulonephritis, Membranous/diagnosis/*etiology ; Humans ; Nephrotic Syndrome/diagnosis/*etiology ; Orientia tsutsugamushi/immunology ; Scrub Typhus/*complications/diagnosis/drug therapy/microbiology ; Treatment Outcome

Allogeneic stem cell transplantation (allo-SCT) using related or unrelated donor could eradicate paroxysmal nocturnal hemoglobinuria (PNH) clones and may cure the disease. Chronic graft-versus host disease (GVHD) is a major complication of patients who have undergone allo-SCT. Nephrotic syndrome has been described as one of the rare manifestations of chronic GVHD following the usual myeloablative allo-SCT. We report a case of nephrotic syndrome that developed 25 months after non-myeloablative allo-SCT for PNH. The patient had grade II acute GVHD and extensive chronic GVHD after non-myeloablative allo-SCT. Typically the patient presented with preserved renal function and full nephrotic syndrome including generalized edema, proteinuria, hypoalbuminemia, and hypercholesterolemia. Renal biopsy revealed findings of membranous glomerulopathy (MG). The patient is alive with a stable engraftment and full donor chimerism under the administration of tacrolimus for control of chronic GVHD and MG without refractory hemolysis and cytopenia.
Adult ; Diagnosis, Differential ; Female ; Glomerulonephritis, Membranous/drug therapy/*etiology/pathology ; Graft vs Host Disease/drug therapy/*etiology/pathology ; Hemoglobinuria, Paroxysmal/*therapy ; Human ; *Stem Cell Transplantation/*adverse effects ; Treatment Outcome

Various forms of hypogammaglobulinemia can occur in patients with autoimmune diseases and vice versa. We report a 13-yr-old boy with membranous nephropathy and common variable immunodeficiency. He presented with the nephrotic syndrome, pneumonia with bronchiectasis, and profound hypogammaglobulinemia. Renal biopsy showed diffusely thickened glomerular capillary walls with 'spikes' suggesting a membranous nephropathy. Secondary causes were ruled out by laboratory studies; however, heavy proteinuria persisted with steroid therapy. Cyclosporine and intravenous immunoglobulin were added, and the patient was discharged with decreased proteinuria. Hypogammaglobulinemia may have a deleterious impact on the immune dysregulation in some patients with membranous nephropathy.
Adolescent ; Bronchiectasis/etiology ; Common Variable Immunodeficiency/complications/*diagnosis/drug therapy ; Cyclosporine/therapeutic use ; Drug Therapy, Combination ; Glomerulonephritis, Membranous/complications/*diagnosis/drug therapy ; Humans ; Immunoglobulins/therapeutic use ; Immunosuppressive Agents/therapeutic use ; Injections, Intravenous ; Kidney/pathology ; Male ; Pneumonia/etiology ; Proteinuria/etiology ; Steroids/therapeutic use