Tubulointerstitial nephritis complicating IVIG therapy for X-linked agammaglobulinemia

BACKGROUND: Patients with X-linked agammaglobulinemia (XLA) develop immune-complex induced diseases such as nephropathy only rarely, presumably because their immunoglobulin (Ig) G concentration is low. We encountered a patient with XLA who developed tubulointerstitial nephritis during treatment with intravenous immunoglobulin (IVIG). CASE PRESENTATION: A 20-year-old man was diagnosed with XLA 3 months after birth and subsequently received periodic γ-globulin replacement therapy. Renal dysfunction developed at 19 years of age in association with high urinary β2-microglobulin (MG) concentrations. A renal biopsy specimen showed dense CD3-positive lymphocytic infiltration in the tubulointerstitium and tubular atrophy, while no IgG4-bearing cell infiltration was found. Fibrosclerosis and crescent formation were evident in some glomeruli. Fluorescent antibody staining demonstrated deposition of IgG and complement component C3 in tubular basement membranes. After pulse steroid therapy was initiated, urinary β2-MG and serum creatinine concentrations improved. CONCLUSION: Neither drug reactions nor collagen disease were likely causes of tubular interstitial disorder in this patient. Although BK virus was ruled out, IgG in the γ-globulin preparation might have reacted with a pathogen present in the patient to form low-molecular-weight immune complexes that were deposited in the tubular basement membrane

A case of systemic lupus erythematosus with Evans syndrome occurring after the remission of minimal change nephrotic syndrome

Evans syndrome is defined as the concomitant or sequential association of autoimmune hemolytic anemia with immune thrombocytopenia, and less frequently autoimmune neutropenia. Here, we encountered a female patient who developed Evans syndrome associated with systemic lupus erythematosus (SLE) during follow-up for minimal change nephrotic syndrome (MCNS). The patient was diagnosed with MCNS at 14 years old. At 17 years old, she came to our hospital with fever, headache, and conjunctival icterus. Blood tests revealed hemolytic anemia, thrombocytopenia, and acute kidney injury. The direct Coombs test was positive, meeting six criteria for the diagnosis of SLE by the American College of Rheumatology. The patient was diagnosed with Evans syndrome secondary to SLE. The renal dysfunction and hematological abnormalities rapidly improved with the administration of prednisolone. Renal biopsy showed more than half of the glomeruli with endocapillary proliferation and wire-loop lesions. Some glomeruli showed segmental sclerosis. Immunofluorescence disclosed granular deposits of IgG, IgM, IgA, C3, and C1q in the glomerular basement membrane and mesangial region. Electron microscopy revealed dense electron deposits in the glomerular vascular endothelium. The patient was further diagnosed with Class IV-S (A/C)-type lupus nephritis and was treated with tacrolimus, with no relapse. Autoimmune disorders of the patient may be involved in the development of nephrotic syndrome. An interesting aspect of this case was the common pathogenesis of SLE and Evans syndrome, which are autoimmune diseases. In addition to the immunological disorders of the host, the influence of external factors may have contributed to the pathogenesis.departmental bulletin pape

〈Case Reports〉A rare case of a severe ocular complication as an initial presentation of adolescent-onset systemic lupus erythematosus: a case report

[Abstract]Background: Systemic lupus erythematosus (SLE) is an autoimmune disease that causes severe complications in multiple organs. Ocular manifestations are critical in patients with SLE because of the vision-threatening risk of such complications. However, severe retinopathy as an initial presentation of SLE is uncommon, even when the severity of SLE is mild, especially in children. Case presentation: We encountered a 14-year-old female patient with rapidly progressive impairment of her left vision that was determined to be caused by severe retinopathy secondary to SLE. Her other systemic symptoms, including a mild renal disorder, were not severe. The patient showed positivity for anticardiolipin antibody, although she did not have antiphospholipid antibody syndrome. She was initially treated with high-dose methylprednisolone, antiplatelet therapy,and anticoagulation therapy because of the high possibility of vision loss; this was followed by tacrolimus as maintenance therapy. Photocoagulation therapy was also performed to prevent vitreous hemorrhage and retinal detachment. Early diagnosis of SLE based on the rapid deterioration of her vision allowed for early interventions and a good clinical course with recovery of her vision. Conclusions: Importantly, regardless of the severity of the systemic symptoms in patients with SLE, ocular involvement is critical and requires aggressive treatment

〈Case Reports〉A patient with renal hypouricemia and acute renal failure after exercise in whom URAT1 gene mutation was demonstrated

〈Abstract〉Patients with the disease entity termed acute renal failure with severe loin pain and patchy renal ischemia after anaerobic exercise (ALPE) manifests non-oliguric renal failure, usually accompanied by few urinary abnormalities such as hematuria and proteinuria. This condition, diagnosed by the demonstration of hypouricemia in addition to these features, usually responds well to conservative treatment. However, differentiation from other renal disorders occasionally proves difficult, which can lead to unnecessary steroid administration and renal biopsy. In the patient reported here, acute glomerulonephritis (AGN) was initially suspected based on his course and urinalysis findings, although typical AGN symptoms such as oliguria, edema, and hypertension were absent. After the detection of hypouricemia, renal hypouricemia was added to the clinical picture. URAT1 gene mutation (W258X, homozygote) was demonstrated by gene analysis. The patient\u27s mother was heterozygous for mutation at the same site