Yoshinosuke Shimamura

Tubulointerstitial nephritis and uveitis syndrome complicated by IgA nephropathy and Graves' disease: a case report

IntroductionTubulointerstitial nephritis and uveitis syndrome is a disorder characterized by a combination of acute tubulointerstitial nephritis and uveitis. Immunoglobulin A nephropathy is defined by the presence of immunoglobulin A deposits in glomerular mesangial areas. In this report, we describe a rare case of tubulointerstitial nephritis and uveitis syndrome complicated by immunoglobulin A nephropathy and Graves’ disease, which was successfully treated with corticosteroids. To the best of our knowledge, this is the first time such a case has been documented since tubulointerstitial nephritis and uveitis syndrome was first described.Case presentationA 64-year-old Japanese woman presented with tubulointerstitial nephritis and uveitis syndrome accompanied by immunoglobulin A nephropathy and Graves’ disease. She had renal dysfunction, proteinuria, and hematuria. Two weeks after her admission, she developed anterior chamber uveitis. She received corticosteroids, resulting in significant clinical improvement.ConclusionTubulointerstitial nephritis and uveitis syndrome is a relatively uncommon cause of tubulointerstitial nephritis. Clinicians should recognize that tubulointerstitial nephritis and uveitis syndrome with immunoglobulin A nephropathy can occur in the presence of Graves’ disease. Additionally, this report may provide important clues in terms of the management of a concomitant case of these diseases.

Tick bite-Erythema migrans

This manuscript describes the typical presentation of erythema migrans caused by tick bite. The purposes of this article are (i) to encourage physicians to observe patients with tick bite carefully since it may cause fatal encephalitis, and (ii) to facilitate physicians, especially in endemic areas, to consider tick‐borne encephalitis virus as one of the causes of encephalitis.

Bevacizumab-induced thrombotic microangiopathy and nephrotic syndrome

A 64-year-old Japanese man with stage 4 adenocarcinoma of the lungs was referred to nephrology for proteinuria 1 month after initiating bevacizumab. The patient was alert and his blood pressure was 133/94 mmHg. There was pretibial edema on both legs. Hemoglobin level was 10.5 g/dL, platelet count was 10.8 × 109/L, and the direct Coombs test was negative. Urinalysis showed 7.6 g/day of proteinuria and 50–99 erythrocytes per high-power field. Renal biopsy showed endotheliosis (Fig. 1), electron-dense deposits in subendothelial areas and foot process effacement (Fig. 2). Immunofluorescence microscopy showed IgA deposition mainly along capillary walls (Fig. 3). Bevacizumab was stopped, and the patient was followed up supportively. The proteinuria and hematuria gradually decreased 6 months thereafter. Bevacizumab, a humanized monoclonal antibody against vascular endothelial growth factor (VEGF), causes thrombotic microangiopathy by reducing VEGF signaling, and nephrotic syndrome due to foot process effacement [1–4]. There is a case that reported electron-dense deposits decreased after the cessation of bevacizumab [5], but the relationship between anti-VEGF antibody and electrondense deposit formation is unclear. Physicians should recognize these complications because they may progress to complete glomerulosclerosis without regression [3].

Diagnostic utility of 18FDG-PET/CT for ADPKD cyst infection

A 63-year-old female on dialysis due to autosomal-dominant polycystic kidney disease (ADPKD) presented with fever of 39 C and left flank pain for 1 week, suggestive of renal cyst infection. Non-contrast computed tomography (Fig. 1a) was not able to identify the infected cyst, but 18-F-fluorodeoxyglucose positron emission computed tomography (F-FDG-PET/CT) revealed an uptake in a renal cyst of the left kidney (Fig. 1b). After ciprofloxacin was initiated, the cyst puncture and drainage were performed. Since cefazolin-sensitive Escherichia coli was found in the fluid culture, ciprofloxacin was adjusted to cefazolin, and completed 4 weeks of the antibiotic therapy. 30–50% of patients with ADPKD experience kidney infection [1], but the accurate diagnosis has been challenging as the utility of computed tomography and magnetic resonance imaging are limited [2]. The higher sensitivity of F-FDG-PET/CT has been reported for the diagnosis of ADPKD cyst infections [3, 4]. Along with appropriate antibiotics [5], early drainage is recommended in large infected cysts [6].

Immunoglobulin A nephropathy secondary to Wilson’s disease: a case report and literature review

Immunoglobulin A nephropathy is the most common primary glomerulonephritis worldwide, and it can be associated with liver disease. However, cases of Immunoglobulin A nephropathy secondary to Wilson’s disease are very rare. A 20-year-old Japanese man presented with microscopic hematuria, proteinuria, and renal dysfunction. A renal biopsy showed mesangial cell proliferation, immunoglobulin A deposition, and electron-dense deposit in the mesangial areas, all of which are consistent with Immunoglobulin A nephropathy. Computed tomography of the abdomen showed liver atrophy and splenomegaly, and the diagnosis of Wilson’s disease was confirmed with decreased serum ceruloplasmin levels, increased urinary copper excretion, Kayser–Fleischer rings and copper deposition in the liver biopsy. The patient was treated successfully with trientine hydrochloride and zinc acetate and showed improvement in renal manifestations. Wilson’s disease is a rare cause of secondary Immunoglobulin A nephropathy. We recommend that Wilson’s disease should be considered the cause of secondary Immunoglobulin A nephropathy in juvenile patients with hematuria, proteinuria, and splenomegaly and suggest measuring the serum ceruloplasmin concentrations, urinary copper excretion, and evaluating Kayser–Fleischer rings in these patients.

Anti-glomerular basement membrane disease accompanied by systemic lupus erythematosus presenting central nervous system involvement

We report a case of rapidly progressive glomerulonephritis caused by anti-glomerular basement membrane (GBM) disease accompanied by systemic lupus erythematosus (SLE) presenting central nervous system involvement in a 32-year-old Japanese male. He was admitted to our hospital because of a 3-week history of fever and rapidly failing renal function requiring hemodialysis (HD). Laboratory tests showed anti-GBM antibody elevation with a value of 16,385 units/ml. On day 85, he had generalized tonic–clonic seizure. Brain magnetic resonance T2 Flair imaging showed multiple high intensity lesions in a broad area. We made a diagnosis of central nervous system involvement in SLE based on positivity for antinuclear and anti-DNA antibodies, hypocomplementemia, and discoid skin rash. After combined therapy consisting of plasma exchange, HD, and steroid pulse, the patient made a good recovery without any residual neurological sequelae, though kidney dysfunction requiring maintenance HD remained. Anti-GBM antibody finally became undetectable on the 144th hospital day.

Hereditary Spherocytosis Presenting with Immunoglobulin A Nephropathy in Post-Splenectomy

Hereditary spherocytosis is a familial hemolytic anemia. In this report, we describe a rare case of hereditary spherocytosis complicated by IgA nephropathy which was successfully treated with an angiotensin converting enzyme inhibitor. This is the first time that such a case in an adult has been documented. We describe a 72‐year‐old Japanese male who presented with hereditary spherocytosis, accompanied by Immunoglobulin A nephropathy. The patient had renal dysfunction, proteinuria, and hematuria. The patient was treated with an angiotensin converting enzyme inhibitor, resulting in clinical improvement. Clinicians should recognize that hereditary spherocytosis with IgA nephropathy can occur in the post‐splenectomy patients.

Renal arteriovenous fistula.

A 65-year-old Japanese male with hypertension presented with flank bruit and deterioration of kidney function. He was performed a percutaneous kidney biopsy in his early twenties for proteinuria (etiology unknown). Enhanced computed tomography revealed enhanced renal vascular malformation, seen as a vascular-attenuation mass on arterial phase (Fig. 1a). Selective renal angiography was performed, which demonstrated an arteriovenous fistula (AVF) and pseudo-aneurysm in the right kidney (Fig. 2). The feeding artery was embolized, and the post-embolization angiogram showed complete obliteration of the fistula (Fig. 3). The flank bruit disappeared after the procedure. Renal AVF usually results from percutaneous renal biopsy, and the incidence has been reported as 18 %. Hypertension and high-output heart failure are major complications because the arteriovenous shunt steals blood flow away from the renal parenchyma [1]. It is diagnosed by the catheter angiography [2], and angiographic embolization is often used as it has fewer complications [3]. Fig. 1 Enhanced computed tomography scan (arterial phase) showing enhanced renal vascular malformation, seen as a vascularattenuation mass

Watermelon stomach: gastric antral vascular ectasia

A 64-year-old Japanese female with transfusion-dependent anemia and chronic kidney disease (CKD) due to diabetic nephropathy presented with upper gastrointestinal bleeding. Red blood cell count was 165 9 10/lL, and hemoglobin level was 6.7 g/dL. Endoscopy revealed red tortuous ectatic vessels along the longitudinal folds of the antrum (Fig. 1), which was characteristic of gastric antral vascular ectasia (GAVE). The patient was treated successfully with three sessions of argon plasma coagulation (APC) (Fig. 2). Her hemoglobin level improved to 10.2 g/dL after the treatment, which helped abolish transfusion dependence, and she had no relapse of symptoms. GAVE accounts for up to 4 % of non-variceal upper gastrointestinal bleeding [1], and is associated with CKD, autoimmune connective tissue diseases, bone marrow transplantation [2], and renal transplantation [3]. APC is the first-line treatment because it is more effective than pharmacological treatment, and leads to abolish transfusion requirements in 77 % of patients [4]. Fig. 1 Initial endoscopy showed tortuous ectatic vessels along the longitudinal folds of the antrum

Lymphoproliferative disease-related mixed cryoglobulinemia treated with rituximab and prednisolone

AbstractMixed cryoglobulinemia is often associated with hepatic C virus infection and is less common with hepatitis B virus infection, and it often progresses into lymphoproliferative diseases. Rituximab is known to achieve systemic B-cell depletion and clinical remission of the systemic effects of cryoglobulins in hepatitis C virus-associated cryoglobulinemia. Conversely, there are few reports regarding the use of rituximab in hepatitis B virus-associated cryoglobulinemia. We report here the case of a 65-year-old Japanese female who presented with lymphoproliferative disease-related cryoglobulinemia with hepatitis B virus, including membranoproliferative glomerulonephritis with renal failure. The vasculitis was refractory to conventional and antiviral therapy, but rituximab use led to control the disease. Our case highlights the benefit and efficacy of rituximab in association with antiviral therapy in small vessel vasculitis related to lymphoproliferative disease-related cryoglobulinemia with hepatitis B virus.