Kazuhiro Hatta

Significantly rapid relief from steroid-resistant nephrotic syndrome by LDL apheresis compared with steroid monotherapy

Rapid amelioration of hypercholesterolemia by LDL apheresis (LDL-A) was performed for long-standing nephrotic syndrome (NS) with hyperlipidemia due to focal segmental glomerulosclerosis (FGS) and the clinical data and prognosis were compared between LDL-A-treated and nontreated groups. Seventeen steroid-resistant NS patients treated with LDL-A (LDL-A group) and 10 NS patients treated with steroids only (steroid-monotherapy (SM) group) were compared. Serum cholesterol and phospholipid levels were significantly lowered only in the LDL-A group (p < 0.01, respectively). The LDL-A group showed a significant decrease of urinary protein (UP, p < 0.01) and increase of serum albumin (p < 0.05). Average time needed to achieve a decrease of UP to less than nephrotic range (< 3.5 g/day) was significantly shorter in the LDL-A group than in the SM group (p < 0.01). Although this is not a prospective study, it is highly expected that a rapid improvement of hypercholesterolemia by LDL-A in steroid-resistant NS will provide more rapid relief from NS than steroid therapy alone.

Radiological features and therapeutic responses of pulmonary nontuberculous mycobacterial disease in rheumatoid arthritis patients receiving biological agents: a retrospective multicenter study in Japan

ObjectiveThis study was performed to evaluate the radiological features of and therapeutic responses to pulmonary disease caused by nontuberculous mycobacteria (NTM) in the setting of biological therapy for rheumatoid arthritis (RA).MethodsWe conducted a retrospective chart review of 13 patients from multiple centers who had developed pulmonary NTM disease during biological therapy for RA, including infliximab, etanercept, adalimumab, and tocilizumab.ResultsMost cases were asymptomatic or resulted in only common-cold-like symptoms. Abnormalities in computed tomography (CT) imaging were protean and frequently overlapped. The most predominant pattern was nodular/bronchiectatic disease (six cases), followed by alveolar infiltrate (three cases), cavitary disease (two cases), and pulmonary nodules (two cases). In most cases, pulmonary NTM disease had spread from a preexisting lesion; in particular, bronchial/bronchiolar abnormalities. In three cases, one or more nodular lesions with or without calcification were a focus of disease. Following the discontinuation of biological agents, most patients responded to anti-NTM therapy. Two patients showed no exacerbation in the absence of any anti-NTM therapy. In one patient, restarting tocilizumab therapy while continuing to receive adequate anti-NTM therapy produced a favorable outcome. In two other patients with a previous history of pulmonary NTM disease, introducing biological therapy led to recurrence, but anti-NTM therapy was effective in these patients.ConclusionCT abnormalities of pulmonary NTM disease in RA patients receiving biological therapy were variable, but were not unique to this clinical setting. NTM disease can spread from preexisting structural abnormalities, even if they are minute. Contrary to our expectations, the therapeutic outcomes of pulmonary NTM disease were favorable in these patients.

Long-term safety and efficacy of rituximab in 7 Japanese patients with ANCA-associated vasculitis.

Abstract Objectives. The safety and efficacy of rituximab were examined in a multicenter open-label pilot study in patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in Japan. Methods. Patients with refractory AAV were administered a rituximab infusion at a weekly dose of 375 mg/m2 for 4 weeks. All patients also received oral daily prednisolone. The primary outcome was complete remission, which was defined as a Birmingham Vasculitis Activity Score (BVAS) of 0 or 1. Results. The mean age of the 7 patients was 57 (range, 34–71) years. The mean follow-up period after rituximab treatment was 62.9 (range, 4.8–81) months. The mean BVAS at entry was 16.7 (range, 2–34). Complete remission occurred in all cases, except in 1 case in which the patient died, with a significant decline in BVAS from baseline at 12 months after initiation of rituximab. Rituximab reduced granulomatous orbital involvement in a patient with granulomatosis with polyangiitis. Relapse occurred in five patients. Adverse events included de novo hepatitis B in one patient, cancer (hepatocellular carcinoma and prostate cancer) in two patients, and transient visual disturbance, atypical mycobacterial infection, urinary tract infection, sepsis, and cytomegalovirus infection. Two patients died due to recurrent infections and airway obstruction, caused by an AAV lesion. Conclusions. Rituximab had a beneficial effect on refractory AAV in Japanese patients, but several adverse effects occurred during rituximab treatment.

Acquired Generalized Anhidrosis: Review of the Literature and Report of a Case with Lymphocytic Hidradenitis and Sialadenitis Successfully Treated with Cyclosporine

We report a case of acquired generalized anhidrosis successfully treated with cyclosporine. A skin biopsy showed T cell infiltration around the sweat glands and labial biopsy revealed lymphoplasmacytic infiltration around the minor salivary gland, suggesting an underlying autoimmune disease such as Sjögrens syndrome. Administration of cyclosporine markedly improved the patients condition and sympathetic skin response; thus cyclosporine may be effective for treating anhidrosis in patients with autoimmune disorders.

Hypereosinophilic syndrome as a cause of fatal thrombosis: two case reports with histological study

Herein we present two cases of hypereosinophilic syndrome with a unique clinical presentation. One patient showed severe systemic thrombosis with splenic rupture and the other patient showed finger gangrene with various systemic symptoms. Both patients were examined histologically, and several characteristics were noted. First, fresh or organized thrombosis with marked eosinophilic infiltration was observed in the cavity and walls of the thrombosed vessels. Second, many eosinophils showed degranulation and were positive for eosinophilic cationic protein on immunohistological examination. Third, the structures of thrombosed vessels were well preserved, which is not observed in systemic vasculitis. These patients exhibited no neoplastic features and were treated with prednisolone with excellent therapeutic results.

Aortic prosthetic graft infection accompanied with esophagomediastinal fistulas: A case report

Prosthetic graft infection is difficult to diagnose early, and hence, is associated with high mortality and morbidity rates. A 63-year-old man who had undergone surgical prosthetic replacement for an inflammatory thoracic aortic aneurysm 10 months previously visited our emergency room, complaining of chills, shivering, frequent vomiting, and back pain. He was diagnosed with severe sepsis, and a blood culture detected Streptococcus anginosus and Prevotella oralis. Repeated contrast-enhanced computed tomography (CT) scans of his chest revealed ectopic gas around the graft, and esophagogastroduodenoscopy revealed esophageal perforations at several sites. We therefore diagnosed him with aortic prosthetic graft infection accompanied with esophagomediastinal fistulas. He received medical treatment and three operations and recovered from the infection. This is a rare case of aortic prosthetic graft infection accompanied with esophagomediastinal fistulas, and we conclude that repeated CT is useful for identifying the primary infection site and invasion route in patients with suspected aortic prosthetic graft infection.

Membranous glomerulonephritis with an LMNA mutation

We had encountered the case of membranous glomerulonephritis (MGN) with dilated cardiomyopathy due to LMNA gene mutation. LMNA mutation was known as a cause of ‘laminopathy’ such as dilated cardiomyopathy, muscular dystrophy, neuropathy and so on. LMNA gene might be a candidate of genetic basis in cryptogenic MGN.

Membranous Nephropathy with Glomerular IgG4 Deposition Without Tubulointerstitial Nephritis in a Patient with Typical IgG4-Related Pancreatic, Hepatic, and Lymph Node Lesions

In 1986, a 45-year-old man, who had a past medical history of swollen submandibular lymph nodes and colon cancer, was admitted for an evaluation of eyelid swelling and generalized edema. On admission, in addition to massive proteinuria, increased total protein (9.2 g/dL) and low albumin (2.7 g/dL) were noted. By several radiological and endoscopic examinations, a diagnosis of chronic pancreatitis was made. And the finding of multiple peritoneal lymph nodes raised the possibility of a cancer recurrence. An open biopsy of several involved organs was performed. The diagnostic conclusions in 1986 were as follows: (1) reactive follicular hyperplasia (lymph node); (2) reactive hepatitis; (3) chronic pancreatitis; and (4) idiopathic membranous nephropathy (MN). These findings were considered to represent a state of hyperimmune reactivity, but no precipitating factor could be identified at that time. When the diagnosis was revisited 25 years later, reinterpretation of the histology, performance of IgG4 staining, and recognition of the features of IgG4-RD in other organs led to reclassification of the patient’s diagnosis as IgG4-related MN without tubulointerstitial nephritis.

A case of effusive–constrictive pericarditis accompanying rheumatoid arthritis: The possibility of adverse effect of TNF-inhibitor therapy

A 68-year-old female, suffering from rheumatoid arthritis, was admitted to our institution for right heart failure with massive pericardial effusion. Her pericardial effusion had increased after starting infliximab, tumor necrosis factor (TNF)-inhibitor therapy, despite improvement in arthralgia. Hemodynamic findings demonstrated effusive-constrictive pericarditis. Because association between exacerbation of pericarditis and infliximab was highly suspected through her clinical course, its administration was stopped. We should pay much attention to pericardial effusion and symptoms of right heart failure after starting TNF-inhibitor therapy in patients with rheumatoid arthritis. <Learning objective: TNF-inhibitor therapy has become a treatment option for rheumatoid arthritis, however, it may cause exacerbation of extra articular manifestations such as pericarditis. We encountered a patient with rheumatoid arthritis who developed effusive-constrictive pericarditis while on TNF-inhibitor therapy. We should pay much attention to pericardial effusion and symptoms of right heart failure after starting TNF-inhibitor therapy in patients with rheumatoid arthritis.>.

Renal biopsy cases in myeloproliferative neoplasms (MPN)

We performed renal biopsy in three cases complicated by myeloproliferative neoplasms (MPN). Although several cases of glomerulonephritis associated with MPN have been reported, the etiologies of the renal disorders were not established (Plomley et al., Aust NZ J Med, 13:125–129, 1983; Sharma et al., Nephron, 69:361, 1995; Kanauchi et al., Intern Med, 33:36–40, 1994; Kasuno et al., Nephrol Dial Transplant, 12:212–215, 1997; Au et al., Am J Kid Dis, 34:889–893, 1999; Kosch et al., Nephrol Dial Transplant, 15:1710–1711, 2000; Oymak et al., Nephron, 86:346–347, 2000; Chun et al., Am J Nephrol, 20:344–346, 2000; Chung et al., Am J Nephrol, 22:397–401, 2002; Asaba et al., Clin Exp Nephrol, 7:296–300, 2003; Haraguchi et al., Clin Exp Nephrol, 10:74–77, 2006; Saigusa et al., J Nephrol, 19:656–658, 2006; Okuyama et al., Clin Nephrol, 6:412–415, 2007; Nishi et al., Clin Nephrol, 5:393–398, 2010; Ulusoy et al., Intern Med, 49:2477, 2010). A review of previous reports of renal biopsy cases with MPN in the English literature suggested that circulation control is important for the treatment of renal disorders that mimic glomerulonephritis in MPN.