We report a case of Churg-Strauss syndrome (CSS) complicated by severe cardiac failure and peripheral neuropathy. Two courses of methylprednisolone pulse therapy were unable to control the disease activity. Repeated intravenous administration of high-dose human immunoglobulin (IVIg) was added together with intravenous cyclophosphamide pulse therapy (IVCY), and the patient's cardiac function and neurological symptoms were gradually ameliorated without any adverse event. Although glucocorticoid and cyclophosphamide comprise the standard therapy for patients with CSS, a number of patients with severe complications appear to be resistant to such conventional treatment. IVIg is thought to be an effective therapeutic option for such patients.
Hypogammaglobulinemia is a reduction or absence of immunoglobulin, which may be congenital or associated with immunosuppressive therapy. In addition to infectious diseases, autoimmune diseases have also been reported in patients with hypogammaglobulinemia. A 26-year-old man with hypogammaglobulinemia had multiple joint pain and swelling with erosive changes in the proximal interphalangeal joint of the right middle finger on X-ray film, mimicking rheumatoid arthritis (RA). As polyarthritis remained after immunoglobulin replacement therapy and there was no finding indicating any infection at that time, a diagnosis of RA was made. Prednisolone and etanercept were started. However, his polyarthritis did not improve and he developed meningitis and massive brain ischemia. Finally, a diagnosis of disseminated Mycoplasma hominis infection was made. The differential diagnosis of polyarthritis in patients with hypogammaglobulinemia should strictly exclude Mycoplasma infection by culture with special media or longer anaerobic culture, and molecular methods for mycoplasma.
SUMMARY: To clarify the characteristics of renal haemodynamics and their correlation with renal pathology in patients with systemic lupus erythematosus (SLE), renal function and renal biopsy findings from 101 SLE patients were analysed retrospectively. Glomerular filtration rate (GFR) and renal plasma flow (RPF) were examined simultaneously. Filtration fraction (FF) was calculated from the values obtained for GFR and RPF (GFR/RPF). The GFR was low in one‐third and within normal limits in two‐thirds of class IV patients with lupus nephritis (LN). In contrast, high RPF was observed in half of class IV patients. As a result, over 70% of class IV patients possessed a very low FF (less than 15%). The sensitivity of very low FF for class IV LN was significantly higher than that of low GFR. In conclusion, low FF was frequently recognized, especially in patients with diffuse proliferative LN. Decreased FF was a highly sensitive indicator of diffuse proliferative LN. Thus, determination of renal haemodynamics, including FF, may be a useful clinical parameter for evaluating renal involvement in patients with SLE.
Sulindac usage may not be associated with an increased incidence of renal stone formation in patients with rheumatoid arthritis Get access Satoshi Ito, Satoshi Ito Department of Medicine, Rheumatic Center of Niigata Prefectural Senami Hospital, 2-4-15 Senami onsen, Murakami-city, Niigata 958-0037, JapanDepartment of Medicine (II), Niigata University School of Medicine, Asahimachi 1, Niigata-city, Niigata 951-8510, Japan Correspondence to: Satoshi Ito, Department of Medicine, Rheumatic Center of Niigata Prefectural Senami Hospital, 2-4-15 Senami onsen, Murakami-city, Niigata 958-0037, Japan Search for other works by this author on: Oxford Academic Google Scholar Hisashi Hasegawa, Hisashi Hasegawa Department of Medicine, Rheumatic Center of Niigata Prefectural Senami Hospital, 2-4-15 Senami onsen, Murakami-city, Niigata 958-0037, JapanDepartment of Medicine (II), Niigata University School of Medicine, Asahimachi 1, Niigata-city, Niigata 951-8510, Japan Search for other works by this author on: Oxford Academic Google Scholar Satoru Nozawa, Satoru Nozawa Department of Medicine, Rheumatic Center of Niigata Prefectural Senami Hospital, 2-4-15 Senami onsen, Murakami-city, Niigata 958-0037, Japan Search for other works by this author on: Oxford Academic Google Scholar Akira Murasawa, Akira Murasawa Department Orthopedic Surgery, Rheumatic Center of Niigata Prefectural Senami Hospital, 2-4-15 Senami onsen, Murakami-city, Niigata 958-0037, Japan Search for other works by this author on: Oxford Academic Google Scholar Masaaki Nakano, Masaaki Nakano Department of Medicine (II), Niigata University School of Medicine, Asahimachi 1, Niigata-city, Niigata 951-8510, Japan Search for other works by this author on: Oxford Academic Google Scholar Masaaki Arakawa Masaaki Arakawa Department of Medicine (II), Niigata University School of Medicine, Asahimachi 1, Niigata-city, Niigata 951-8510, Japan Search for other works by this author on: Oxford Academic Google Scholar Japanese Journal of Rheumatology, Volume 9, Issue 1, 1 March 1999, Pages 119–121, https://doi.org/10.3109/BF03041268 Published: 01 March 1999 Article history Received: 12 May 1998 Accepted: 07 October 1998 Published: 01 March 1999
Amyloidosis is a term applied to a heterogeneous group of rare diseases characterized by extracellular deposition of amyloid, causing target-organ dysfunction and a wide range of clinical symptoms [1].These symptoms depend on the organ involved, and include nephrotic syndrome, hepatosplenomegaly, congestive heart failure, carpal tunnel syndrome, gastrointestinal (GI) symptoms and macroglossia [2].Amyloidosis is clinically classified into several types depending on the precursor of the amyloid fibril.The disease involves amyloid fibrils formed in vivo by 27 different types of protein [3] (Table 1).Reactive amyloid A (AA) amyloidosis is the representative systemic condition that develops in patients with chronic inflammatory diseases such as rheumatoid arthritis (RA), juvenile idiopathic arthritis, ankylosing spondylitis, inflammatory bowel disease, familial periodic fever syndrome, and chronic infections [4,5,6,7].In some parts of the world, heredofamilial causes and infections are responsible for a larger proportion of cases of AA amyloidosis.In Turkey, familial Mediterranean fever (FMF) is the cause of more than 60 percent of cases [8].Other conditions that may be associated with AA amyloidosis include neoplasms, particularly renal cell carcinoma [9], non-Hodgkin lymphoma [10], Castleman's disease [11], and cystic fibrosis [12].Recently, therapy with biologic agents including anti-tumor necrosis factor (anti-TNF) and anti-interleukin-6 (IL-6) is now employed routinely for the management of RA in patients for whom traditional disease-modifying anti-rheumatic drugs (DMARDs) have failed.In parallel with this shift of treatment strategy, the treatment of amyloidosis has also changed.This article discusses current concepts of AA amyloidosis that is mainly secondary to RA, and addresses various strategies for prophylaxis, diagnosis, and therapy of this important complication in the light of changes in clinical management, especially hemodialysis (HD). PrevalenceEpidemiological data for AA amyloidosis, extrapolated from autopsy records in Western nations, has indicated that the prevalence varies from about 0.5% to 0.86% according to environmental risk factors and geographic clustering [13,14].The incidence of AA www.intechopen.comAmyloidosis -Mechanisms and Prospects for Therapy 44 amyloidosis in RA is still undefined, and is considered to be underestimated.In Europe, 5-20% of patients with RA develop amyloidosis, with the highest incidence in Finland [15], where reevaluation of autopsy materials for the period 1952-1991 yielded a 30% incidence of AA amyloidosis compared with 18% detected by routine testing, indicating that a significant proportion of cases may not be detected by standard histologic analysis [16].Japanese autopsy reports have revealed that about 30% of autopsied RA patients have amyloid deposits [17].Some Japanese medical centers have reported the incidence of amyloidosis in consecutive patients undergoing GI biopsy.The frequency of amyloidosis in RA has been reported to vary between 5% and 13.3% in cases confirmed by biopsy, and from 14% to 26% in cases confirmed at autopsy [18,19,20,21].*Proteins are listed, when possible, according to relationship.Thus, apolipoproteins are grouped together, as are polypeptide hormones.*ADan comes from the same gene as ABri.**Also called 'amylin'.RA begins with joint synovitis, and serum amyloid A protein (SAA) is synthesized in the liver chiefly as a result of stimulation with proinflammatory cytokines.Genetic background factors such as the SAA 1.3 allele genotype are a risk factor for amyloidosis.Amyloid fibrils are deposited in tissues of various organs, leading to organ failure.TNF- tumor necrosis factor- IL-6: interleukin-6, IL-1: interleukin-1, SAA1.3: one of the SAA1 gene polymorphisms.
Anti-neutrophil cytoplasmic autoantibodies against bactericidal/permeability-increasing protein (BPI-ANCA) are known to be present in patients with cystic fibrosis, diffuse panbronchiolitis, and inflammatory bowel disease, especially in relation to chronic Gram-negative bacterial infection. To investigate the possible role of BPI-ANCA in rheumatoid arthritis (RA), we measured the serum titer of BPI-ANCA and examined clinical manifestations, including pulmonary complications, in patients with RA. Seventy-four RA patients were recruited to our study. The titer of BPI-ANCA was measured by enzyme-linked immunosorbent assay (ELISA). Pulmonary complications were evaluated using high-resolution computed tomography (HRCT), which revealed 26 patients with bronchial diseases (BD group), 25 with interstitial pneumonia (IP group), and 23 without any particular lung lesion (normal group). The correlations between the titer of BPI-ANCA and patients’ clinical and laboratory findings were then analyzed. The numbers of tender joints, swollen joints, and the Disease Activity Score including 28 joint count were significantly higher in the BD group. The titer of BPI-ANCA was positively correlated with age, erythrocyte sedimentation rate (ESR), and bronchial involvement in all subjects. Stepwise multiple regression analysis of factors affecting the titer of BPI-ANCA selected ESR and bronchial involvement as independent variables. Our results show that BPI-ANCA was positively correlated with chronic inflammatory status in RA patients, and we is believe it is positively linked with bronchial diseases.
Journal Article Anti-neutrophil cytoplasmic autoantibodies against bactericidal/permeability-increasing protein in patients with rheumatoid arthritis and their correlation with bronchial involvement Get access Yoko Wada, Yoko Wada Division of Clinical Nephrology and Rheumatology, Niigata University Graduate School of Medical and Dental Sciences, 1-757 Asahimachi-dori, Niigata 951-8510, Japan Correspondence to: Yoko Wada, Division of Clinical Nephrology and Rheumatology, Niigata University Graduate School of Medical and Dental Sciences, 1-757 Asahimachi-dori, Niigata 951-8510, Japan e-mail: yoko.wada@gmail.com Search for other works by this author on: Oxford Academic Google Scholar Takeshi Kuroda, Takeshi Kuroda Division of Clinical Nephrology and Rheumatology, Niigata University Graduate School of Medical and Dental Sciences, 1-757 Asahimachi-dori, Niigata 951-8510, Japan Search for other works by this author on: Oxford Academic Google Scholar Akira Murasawa, Akira Murasawa Niigata Rheumatic Center, Shibata, Japan Search for other works by this author on: Oxford Academic Google Scholar Masaaki Nakano, Masaaki Nakano School of Health Sciences, Faculty of Medicine, Niigata University, Niigata, Japan Search for other works by this author on: Oxford Academic Google Scholar Ichiei Narita Ichiei Narita Division of Clinical Nephrology and Rheumatology, Niigata University Graduate School of Medical and Dental Sciences, 1-757 Asahimachi-dori, Niigata 951-8510, Japan Search for other works by this author on: Oxford Academic Google Scholar Modern Rheumatology, Volume 20, Issue 3, 1 June 2010, Pages 252–256, https://doi.org/10.3109/s10165-009-0270-1 Published: 01 June 2010 Article history Received: 02 September 2009 Accepted: 15 December 2009 Published: 01 June 2010
