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ID PMID Title PublicationDate abstract
23430383 P wave dispersion in juvenile idiopathic arthritis patients with diastolic dysfunction. 2012 Dec OBJECTIVE: Cardiac involvement as pericarditis, myocarditis and valvular disease is common in juvenile idiopathic arthritis (JIA). However, there are few studies concerning systolic and diastolic functions of the left ventricle in children with JIA. P wave dispersion is a sign for the prediction of atrial fibrillation. A recent study found that rheumatoid arthritis patients had an abnormally high P wave duration and P wave dispersion, markers for supraventricular arrhythmogenicity. In this study, we aimed to evaluate P wave dispersion and its relation with diastolic dysfunction of the left ventricle in patients with JIA. METHODS: We performed electrocardiography and Doppler echocardiography on patients and controls. Maximum and minimum P wave duration were obtained from electrocardiographic measurements. P wave dispersion defined as the difference between maximum and minimum P wave duration was also calculated. FINDINGS: No statistically significant differences were found between the patients and controls in minimum, maximum P wave duration and P wave dispersion. Among the diastolic parameters in patients group, increased late flow velocity, decreased early flow velocity and prolonged isovolumic relaxation time reflected diastolic dysfunction. CONCLUSION: During 12 months of follow-up, no supraventricular arrhythmias were documented in JIA with diastolic dysfunction. JIA with diastolic dysfunction has normal atrial conduction parameters and therefore seemingly do not have an increased risk of atrial fibrillation.
23346400 Expression of Angiotensin II Receptor-1 in Human Articular Chondrocytes. 2012 Background. Besides its involvement in the cardiovascular system, the renin-angiotensin-aldosterone (RAS) system has also been suggested to play an important role in inflammation. To explore the role of this system in cartilage damage in arthritis, we investigated the expression of angiotensin II receptors in chondrocytes. Methods. Articular cartilage was obtained from patients with osteoarthritis, rheumatoid arthritis, and traumatic fractures who were undergoing arthroplasty. Chondrocytes were isolated and cultured in vitro with or without interleukin (IL-1). The expression of angiotensin II receptor types 1 (AT1R) and 2 (AT2R) mRNA by the chondrocytes was analyzed using reverse transcription-polymerase chain reaction (RT-PCR). AT1R expression in cartilage tissue was analyzed using immunohistochemistry. The effect of IL-1 on AT1R/AT2R expression in the chondrocytes was analyzed by quantitative PCR and flow cytometry. Results. Chondrocytes from all patient types expressed AT1R/AT2R mRNA, though considerable variation was found between samples. Immunohistochemical analysis confirmed AT1R expression at the protein level. Stimulation with IL-1 enhanced the expression of AT1R/AT2R mRNA in OA and RA chondrocytes. Conclusions. Human articular chondrocytes, at least partially, express angiotensin II receptors, and IL-1 stimulation induced AT1R/AT2R mRNA expression significantly.
22641595 Autoimmune thyroid disease in patients with rheumatic diseases. 2012 May Thyroid function abnormalities and thyroid autoantibodies have been frequently described in patients with rheumatologic autoimmune diseases, such as Sjögren's syndrome, rheumatoid arthritis, systemic lupus erythematosus and scleroderma. Limited data are available regarding the prevalence and clinical characteristics of autoimmune thyroiditis in other rheumatologic disorders, such as rheumatic fever and juvenile systemic lupus erythematosus. The authors review the association of endocrine autoimmune and rheumatic autoimmune diseases, assessing various age groups and clinical conditions. The bibliographic survey was conducted through the search for scientific articles indexed in the general health sciences databases, such as Latin American and Caribbean Health Sciences Literature (LILACS), Medline/PubMed, and Scientific Electronic Library Online (SciELO). The following descriptors were used: "rheumatic autoimmune diseases and autoimmune thyroid diseases"; "thyroid disorders and rheumatic diseases"; "thyroiditis and rheumatic diseases"; "autoimmune diseases and thyroid"; and "pediatric rheumatic diseases and autoimmune thyroid diseases". This study showed that, despite contradictory results in the literature, there is a greater prevalence of the association between autoimmune thyroid diseases and rheumatic diseases, highlighting the possibility of common pathogenic mechanisms among them.
21816647 Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) and oligoarthriti 2011 Dec A 24-year-old woman who had sinus histiocytosis with massive lymphadenopathy (SHML, Rosai-Dorfman disease) also had oligoarthritis. We found only four previously reported cases of SHML with clinical joint disease. The clinical picture may suggest rheumatoid arthritis or a spondylarthropathy with peripheral joint involvement. SHML should be considered routinely among the differential diagnoses in young patients with arthritis and large lymphadenopathies. There is no consensus regarding the treatment. In our patient, conventional disease-modifying antirheumatic drugs followed by 3 months of adalimumab then 3 months of etanercept had no effect on the symptoms.
21143496 Appropriate infliximab infusion dosage and monitoring: results of a panel meeting of rheum 2011 Jan WHAT IS ALREADY KNOWN ABOUT THIS SUBJECT: Infliximab is an effective treatment for rheumatoid arthritis, ankylosing spondylitis, Crohn's disease (both adult and paediatric), ulcerative colitis, psoriatic arthritis and plaque psoriasis and national and international guidelines have been developed for each indication. WHAT THIS STUDY ADDS: This study is the first study which compared current international, national and local guidelines from the medical specialties involved in the treatment with infliximab on the following topics: indication, dosage, synergy and monitoring of vital signs. AIMS: Infliximab, an anti-TNF biologic agent, is currently indicated and reimbursed for rheumatoid arthritis, ankylosing spondylitis, Crohn's disease (both adult and paediatric), ulcerative colitis, psoriatic arthritis and plaque psoriasis. Development of national and international guidelines for rheumatology, gastroenterology and dermatology, was mostly based on clinical studies and expert opinion. The aim of this study was to compare available guidelines and local protocols for rheumatology, dermatology and gastroenterology, regarding dosage of infliximab, synergy of infliximab with concomitant medication and monitoring of vital signs during infliximab administration, for achieving optimal care. METHODS: Current international, national and local guidelines on the use of infliximab were reviewed and compared, differences and shortcomings were identified, and optimal treatment schedules discussed during a meeting (July 2008) of clinical experts and researchers from three departments of a Dutch university hospital. RESULTS: Recommended dosages of infliximab are not equal for different indications. Loss of response to infliximab is a common problem encountered within the three medical specialties, but indications for adjustments in treatment schedules are lacking in all of the guidelines. Monitoring of vital signs (blood pressure, pulse, temperature) during infusion with infliximab is common practice and recommended by some guidelines. Routine measurement of vital signs is not of any value in predicting or recognizing acute infusion reactions, in our experience, and this is confirmed by literature on inflammatory bowel disease. CONCLUSION: Different indications encompass different dosing schedules. National and internal guidelines do not provide advice regarding loss of response. Routine measurement of vital signs during infusion is not valuable in detecting acute infusion reactions and should only be performed in case of an acute infusion reaction. These topics need to be studied in future studies and covered in future guidelines.
21364053 The value of conventional radiographs in undifferentiated arthritis: a systematic review. 2011 Mar OBJECTIVE: To perform a systematic literature review on the diagnostic and predictive value of conventional radiographs (CR) in patients with undifferentiated arthritis (UA). METHODS: We performed an extended search using Medline, Embase, the Cochrane Library, and abstracts from the 2007 and 2008 meetings of the American College of Rheumatology and the European League Against Rheumatism. Articles were included based on predefined inclusion criteria, and quality was assessed by using validated quality scales. RESULTS: In total, 25 articles were included from 6003 retrieved references. Five articles described a pure UA population, 20 articles described a mixed population [mostly rheumatoid arthritis (RA) and UA]. In studies on UA, erosions on CR were strong predictors of RA diagnosis [positive likelihood ratio (LR+) 3.5-10.9; odds ratio 7.6 and 8.7). In a more heterogeneous mixed population, 20 studies reporting on 11 cohorts found a relationship between CR findings and subsequent diagnosis of RA. LR+ for erosions and/or bony decalcifications ranged from 1.8 to 9.7, and there was greater prevalence of erosions and higher Sharp-van der Heijde score in the RA group at followup. With regard to prognosis in both UA and mixed populations, an association was found between number of abnormalities on CR and poor outcome. CONCLUSION: Several studies, in pure UA and mixed populations, clearly demonstrate that CR are helpful in predicting future diagnosis of RA or worse prognosis. However, absence of abnormalities on CR does not sufficiently exclude RA or other unfavorable outcome.
22751454 Paradoxical inflammation induced by anti-TNF agents in patients with IBD. 2012 Sep Anti-TNF antibodies have acquired a prominent place in the management of IBD (including Crohn's disease and ulcerative colitis), rheumatologic conditions (such as rheumatoid arthritis, ankylosing spondylitis and psoriatic arthritis) and psoriasis. They have a good safety profile, especially when contraindications such as demyelinating disease, active infections and/or abscesses are ruled out, and when necessary precautions to prevent reactivation of tuberculosis are taken. However, with increasing use of these agents, paradoxical adverse events have been reported. Some of these features are shared with the underlying disease for which these drugs are given, making management of these conditions challenging. For example, anti-TNF therapy is used for the treatment of psoriasis, but psoriasiform lesions are sometimes observed in patients receiving therapy. Similarly, anti-TNF therapy is used for the treatment of rheumatologic diseases, but arthralgias and arthritis are sometimes observed in patients receiving anti-TNF agents. We review the paradoxical inflammation induced by anti-TNF agents in patients with IBD, provide hypotheses for the occurrence of this paradoxical inflammation and give practical advice on how to manage these patients.
22127694 Crystal structure of an arthritogenic anticollagen immune complex. 2011 Dec OBJECTIVE: In rheumatoid arthritis, joint inflammation and cartilage destruction are mediated by autoantibodies directed to various self antigens. Type II collagen (CII)-specific antibodies are likely to play a role in this process and have been shown to induce experimental arthritis in susceptible animals. The purpose of this study was to reveal how arthritogenic autoantibodies recognize native CII in its triple-helical conformation. METHODS: Site-directed mutagenesis and crystallographic studies were performed to reveal crucial contact points between the CII antibody and the triple-helical CII peptide. RESULTS: The crystal structure of a pathogenic autoantibody bound to a major triple-helical epitope present on CII was determined, allowing a first and detailed description of the interactions within an arthritogenic complex that is frequently occurring in both mice and humans with autoimmune arthritis. The crystal structure emphasizes the role of arginine residues located in a commonly recognized motif on CII and reveals that germline-encoded elements are involved in the interaction with the epitope. CONCLUSION: The crystal structure of an arthritogenic antibody binding a triple-helical epitope on CII indicates a crucial role of germline-encoded and arginine residues as the target structures.
22382034 Glenohumeral tuberculous arthritis complicated with beta haemolytic streptococcus: An extr 2012 INTRODUCTION: Septic arthritis of the glenohumeral joint is a rare entity and its diagnosis is difficult with a superadded infection in the presence of underlying tuberculosis. We report the first case of group B beta haemolytic streptococcal glenohumeral arthritis with underlying tuberculosis. CASE PRESENTATION: A 40 year old lady previously diagnosed to have poliomyelitis, rheumatoid arthritis, hepatitis C, and diabetes mellitus for the last 10 years, presented to the emergency room with diabetic ketoacidosis. Two weeks prior to presentation she developed fever along with pain and swelling in left shoulder with uncontrolled blood sugars. Local examination of the shoulder revealed global swelling with significant restricted range of motion. MRI showed a large multiloculated collection around the left shoulder joint extending into the axilla, and proximal arm. Urgent arthrotomy performed and about 120ml thick pus was drained. The patient was started on clindamicin and antituberculous chemotherapy and her symptoms dramatically improved. DISCUSSION: Bone and joint involvement accounts for approximately 2% of all reported cases of tuberculosis (TB), and it accounts for approximately 10% of the extra pulmonary cases of TB. Tuberculosis of the shoulder joint constitutes 1-10.5% of skeletal tuberculosis. Classical symptoms of fever, night sweats, and weight loss may be absent, and a concurrent pulmonary focus may not be evident in most cases. CONCLUSION: Despite acute presentation of septic arthritis, in areas endemic for tuberculosis and particularly in an immunocompromised patient, workup for tuberculosis should be part of the routine evaluation.
22274610 The illnesses of Carlo di Ferdinando I de' Medici: a second opinion. 2012 Jan OBJECTIVES: To offer a second opinion on the recently published retrospective diagnosis of Cardinal Carlo de' Medici (1596-1666), a prominent member of the grand ducal family then ruling Tuscany. METHODS: Retrospective diagnosis of historical figures is difficult and at times controversial, even with modern technology. It is based on contemporaneous medical descriptions and historical reviews, inherited iconography, and rarely - as in the case of the Medici of Florence - skeletal assessment, completed with radiological, histological and even immunological studies. Modern clinical work is often complemented with a second opinion obtained from specialists in the relevant fields. It is this type of second opinion that our collaborative Australian and Italian team, comprised of an orthopaedic/spinal surgeon, a rheumatologist and two medical historians, now offers. RESULTS: The authors concur with the first opinion's diagnosis of Klippel-Feil syndrome in Carlo de' Medici, but disagree with the diagnoses of tuberculosis (Pott's disease) and rheumatoid arthritis. We find evidence, instead, for a psoriatic-DISH arthropathy with involvement of Klippel-Feil syndrome. CONCLUSIONS: A psoriatic-DISH arthropathy, previously described by the present authors as the 'Medici syndrome', was commonly found in the males of the primary branch of the family. The diagnosis of this condition in Cardinal Carlo de' Medici represents its first identification in a male of the secondary (grand ducal) branch of the family.
20049449 Evaluation of inflammatory change and bone erosion using a murine type II collagen-induced 2011 May The exact mechanism of rheumatoid arthritis (RA) is unclear, but a combination of genetic, environmental and hormonal factors is thought to be involved. This study examined the progressive arthritic reaction of murine type II collagen-induced arthritis (CIA), a representative animal model of RA. Arthritic reactions, including inflammation and bone erosion were examined using an objective non-invasive method. Two scoring systems were used to evaluate changes in cutaneous inflammation and bone erosion during RA progression. The severity of inflammation was evaluated by visual scoring of erythema and edema, while the degree of bone erosion was quantified by macroradiographical erosion analysis of specific bones. A significant difference was observed in both visual (P = 0.0001, n = 7) and radiographic (P < 0.0001, n = 7) examinations for the RA group as compared to the control. The relationship between inflammatory change and erosive change in bone showed a significant positive correlation, r = 0.9550 (P < 0.0001, n = 7). The overall rate of asymmetry was 25.23% in both fore- and hindpaws. The results generated from these experiments show that murine CIA is a promising model for elucidating the mechanism of RA. In addition, the results of this study may be used for monitoring RA progression as well as screening therapy efficacy in the joint pathology.
23261010 Fatigue and widespread pain in systemic lupus erythematosus and Sjögren's syndrome: sympt 2012 Nov Fatigue and generalised pain are debilitating symptoms that negatively impact the quality of life in patients with systemic lupus erythematosus (SLE) and primary Sjögren's syndrome (pSS). Chronic widespread musculoskeletal pain and fatigue are the clinical hallmarks of fibromyalgia (FM), a clinical entity which can be associated to connective tissue disease. The aim of the present study was to assess the prevalence of FM syndrome, fatigue and widespread pain in SLE and pSS patients and to evaluate the contribution of inflammatory disease and FM on those constitutional symptoms. Fifty SLE and 50 pSS patients were enrolled in the study. Patients rated fatigue, pain, and disease activity using a 100-mm visual analogue scale and completed the Health Assessment Questionnaire and the Fibromyalgia Impact Questionnaire. Zung depression and anxiety scales were used to quantify mood disorders. Tender points were evaluated using an algometer. Disease activity score as evaluated for each SLE and pSS patient. Fibromyalgia has been diagnosed in a significantly higher percentage of SLE patients than pSS patients (32% vs. 18%, p=0.022) even if the percentage of patients reporting fatigue and pain was higher among pSS patients. No correlation with disease activity was observed in either group of patients. FM seems to contribute to constitutional symptoms more in SLE than in pSS, suggesting a different underlying cause of fatigue and widespread pain in these two different connective tissue diseases.
23001257 Prevalence and predictors of Sjogren's syndrome in a prospective cohort of patients with a 2012 Dec AIMS: To assess the prevalence and determine predictors of Sjögren's syndrome (SS) in patients with clinically significant aqueous-deficient dry eye. METHODS: Patients enrolled in an industry-sponsored, multicentre clinical trial (NCT00784719) were assessed prospectively for the presence of SS. Ocular testing included Schirmer test, corneal fluorescein staining, conjunctival lissamine green staining, and tear-film breakup time. Review of systems questionnaire, medical history, dry eye questionnaire and laboratory work-up (Sjögren-specific antibody A (SSA), Sjögren-specific antibody B (SSB), rheumatoid factor (RF) and antinuclear antibody (ANA)) were obtained. RESULTS: Of 327 patients, 38 (11.6%) had SS: 21 (6.4%) with primary SS (pSS), and 17 (5.2%) with secondary SS. Nine patients (3%) were newly diagnosed using the applied diagnostic criteria based on American-European consensus criteria. Patients with SS had significantly worse conjunctival and corneal staining, Schirmer test (with and without anaesthesia), and symptoms compared with patients without SS. pSS Was significantly more likely to occur in patients with positive ANA (OR: 13.9) and RF (OR: 4.8). CONCLUSIONS: Ophthalmologists caring for patients with clinically significant dry eye should have a high index of suspicion for underlying SS and low threshold for serological work-up. RF and ANA are recommended as useful tests in SSA/SSB-negative patients for further diagnostic referral.
22041773 [The effect of cyclophosphamide on cytokines in patients with primary Sjögren's syndrome- 2011 Jul OBJECTIVE: To investigate the mechanisms of cyclophosphamide sequential therapy for patients with primary Sjögren's syndrome-associated interstitial lung disease (PSS-ILD). METHODS: This was a retrospective review of 15 patients (2005 - 2008) with PSS-ILD who underwent cyclophosphamide sequential therapy. Peripheral blood and bronchoalveolar lavage (BALF) were obtain before and 3, 6, 12, 24 months after the treatment. The TNF-α and TGF-β(1)mRNA levels in peripheral blood were measured using reverse transcription-polymerase chain reaction (RT-PCR). Serum and BALF TNF-α, TGF-β(1)and MMP-9 levels were measured using sandwich enzyme-linked immunosorbent assay (ELISA). RESULTS: (1) The average levels of serum TNF-α (0.39 ± 0.22) and TGF-β(1) (0.31 ± 0.18) mRNA in patients with PSS-ILD were higher compared with that in patients with PSS without ILD. TNF-α level (0.23 ± 0.19) was significantly decreased 3 months after cyclophosphamide treatment (t = 2.533, P < 0.05), and TGF-β(1) (0.31 ± 0.18) level markedly decreased after 6 months of treatment (t = 2.617, P < 0.05). (2) The levels of serum TNF-α (11.2 ± 2.6) µg/L, TGF-β(1) (72 ± 19) µg/L and MMP-9 (38 ± 9) µg/L in patients with PSS-ILD were higher than that in patients with PSS without ILD. TGF-β(1) (36 ± 12) µg/L level decreased significantly after 3 months of treatment (t = 2.526, P < 0.05), and TNF-α level (7.1 ± 1.3) µg/L markedly decreased after 6 months of therapy (t = 2.578, P < 0.05). MMP-9 level (18 ± 4) µg/L decreased significantly after 12-month treatment (t = 2.329, P < 0.05). (3) The levels of BALF TNF-α (17.1 ± 3.5) µg/L, TGF-β(1) (36 ± 17) µg/L and MMP-9 (27 ± 10) µg/L in patients with PSS-ILD were higher than that in patients with PSS without ILD. TGF-β(1) (21 ± 14) µg/L level decreased significantly after 3-month treatment, and TNF-α level (9.4 ± 1.7) µg/L was decreased after 6 months of cyclophosphamide treatment (t = 2.215, P < 0.05). MMP-9 level (13 ± 5) µg/L decreased after 12 months of cyclophosphamide treatment (t = 2.576, P < 0.05). CONCLUSION: The mechanisms of cyclophosphamide treatment may be associated with its inhibition on production of TNF-α, TGF-β(1)and MMP-9.
22334275 Treatment of experimental arthritis by targeting synovial endothelium with a neutralizing 2012 Aug OBJECTIVE: To show that a new recombinant protein (MT07) obtained by fusing a synovial-homing peptide to a neutralizing antibody to C5 can be selectively delivered to inflamed synovium and can effectively control joint inflammation in experimental models of arthritis. METHODS: Binding of MT07 to human, rat, and mouse synovial tissue was evaluated in vitro by immunofluorescence, and selective localization in the inflamed joints of rats was documented in vivo using time-domain optical imaging. The antiinflammatory effect of MT07 was tested in a rat model of antigen-induced arthritis (AIA) and in a mouse model of collagen antibody-induced arthritis (CAIA). RESULTS: MT07 was able to bind to samples of inflamed synovium from humans, mice, and rats while failing to recognize uninflamed synovium as well as inflamed mouse lung or rat kidney. In vivo analysis of the biodistribution of MT07 confirmed its preferential homing to inflamed joints, with negligible inhibition of circulating C5 levels. MT07 prevented and resolved established inflammation in a rat model of AIA, as demonstrated by changes in joint swelling, polymorphonuclear cell counts in synovial washes, release of interleukin-6 and tumor necrosis factor α, and tissue damage. A similar therapeutic effect was obtained testing MT07 in a CAIA model. CONCLUSION: Our findings show that the novel recombinant molecule MT07 has the unique ability to selectively target inflamed joints and to exert local control of the inflammatory process by neutralizing the complement system without interfering with circulating C5 levels. We believe that this approach can be extended to other antiinflammatory drugs currently used to treat patients with rheumatoid arthritis.
22163062 Verification of the genomic identity of candidate microchimeric cells. 2011 Jul Microchimerism has been studied in the context of a variety of diseases which include autoimmune diseases (such as systemic sclerosis, rheumatoid arthritis, systemic lupus erythematosus and autoimmune thyroid diseases), cancer (e.g., of the cervix, thyroid gland, lung, breast), tissue repair, transplantation and transfusion. It may become relevant in the context of cell-based non-invasive prenatal diagnosis. But how to safely identify individual microchimeric cells? This is a nontrivial question, for which a solution has recently been suggested.
21946462 Primary sjögren syndrome manifested as localized cutaneous nodular amyloidosis. 2011 Oct Localized cutaneous nodular amyloidosis (LCNA) is the rarest type of cutaneous amyloidosis. Typically presenting as waxy nodules on the lower extremities, it demonstrates localized deposition of AL-type amyloid in immunohistologic study and is often associated with focal plasma cell proliferation. Sjögren syndrome, an autoimmune lymphoproliferative disorder, is characterized by keratoconjunctivitis sicca and xerostomia with lymphocytic infiltration of exocrine glands. As shown in case reports, the association of LCNA with Sjögren syndrome is considerable. Herein, we report a 78-year-old woman with LCNA, who was further surveyed and diagnosed with Sjögren syndrome. In light of the significant relation between these 2 diseases, further examination for coexistence of Sjögren syndrome in addition to systemic amyloidosis is well warranted. Prompt identification of an underlying Sjögren syndrome in LCNA with polyclonal immunoglobulin amyloid may have important therapeutic consequences.
21394665 Long-term remission of pulmonary veno-occlusive disease associated with primary Sjögren's 2011 Dec The patient described here is a 21-year-old Japanese woman with primary Sjögren's syndrome (pSS) presenting with worsening of dyspnea, palpitation, recurrent parotitis, and arthritis. Chest computed tomography showed diffuse interlobular septal thickening and ground-glass opacities. Right heart catheterization demonstrated pulmonary hypertension, right-sided heart failure, normal pulmonary capillary wedge pressure, and no evidence of arterio-venous shunt. Transbronchial lung biopsy showed luminal obliteration of pulmonary venules by intimal cellular proliferations, without abnormalities in the small pulmonary arteries. These findings were consistent with pulmonary veno-occlusive disease (PVOD). Immunosuppressive therapy, starting with prednisolone 20 mg/day and subsequently combined with azathioprine, resulted in the disappearance of the signs and symptoms, including exertional dyspnea and abnormal pulmonary parenchymal shadows on computed tomography, and the normalization of pulmonary artery pressure. So far, there have been no reported cases of PVOD associated with pSS. Of interest, immunosuppressive therapy without vasodilator therapy almost completely resolved the pulmonary hypertension in this patient.
22766490 [A rare pulmonary lesion association]. 2012 Oct INTRODUCTION: Pulmonary amyloidoma or nodular amyloidosis is a localized form of amyloidosis, which can mimic a bronchopulmonary carcinoma. This form of amyloidosis may be associated to an infectious, a systemic disease or a lymphoma. OBSERVATION: We describe the case of a 36-year-old patient whose past medical history was consistent for a diabetes mellitus and a hypothyroidism treated by medical treatment. The patient presented a Gougerot-Sjögren syndrome and was explored for non-specific respiratory symptoms. Physical examination was normal. Laboratory tests revealed a monoclonal pic of immunoglobulin. Radiologic findings showed bilateral pulmonary nodules associated to mediastinal lymph nodes. A pulmonary biopsy was performed. Histologic examination revealed a tumoral nodule containing an abundant eosinophilic material, which was acellular and surrounded by a dense lymphomatous infiltrate destroying the pulmonary parenchyma. Histochemical and immunohistochemical study revealed an association of a nodular pulmonary amyloidosis with a MAT pulmonary lymphoma complicating a Gougerot-Sjögren syndrome. CONCLUSION: The association of MALT pulmonary lymphoma and localized amyloidosis is rarely observed in case of Gougerot-Sjögren syndrome. The pathogenesis of this association remains unknown and the management non-consensual because of the rarity of the cases reported. Whereas, it appears that localized amyloidosis associated to a MALT lymphoma seems to have a better prognosis than a disseminated amyloidosis.
21964047 Implications of long-term medication of oral steroids and antimalarial drugs in primary Sj 2011 Dec BACKGROUND: Immunomodulating drugs are commonly used in treating patients with autoimmune diseases but with very different outcomes. We aimed to investigate differences in cytokine and autoantibody levels with regard to patient characteristics in patients with primary Sjögren's syndrome (pSS) receiving oral steroids or antimalarial drugs (AM) after a longer period of time. METHODS: Serum samples from 141 patients fulfilling the revised EU-US criteria and 99 healthy controls were analysed for 25 cytokines and 8 autoantibodies. RESULTS: AM-patients had lowered levels of IL-5, IL-10, IL-13 and IFN-γ, though non-significantly. Use of prednisolone was associated with reduced levels of IL-15, IL-2, IL-4, IL-12p40, TNF-α, MIP-1α and MIP-1β (p<0.05), and a trend towards decreased levels of IL-1RA and IL-1β was observed. No associations were seen between AM and antibody levels. Significantly higher protein levels of anti-Ro-52 and anti-Ro-60 were observed in the patients taking prednisolone (p<0.05). The proportion of patients positive for anti-Ro-52 and anti-La-48 did not differ significantly in the groups taking and not taking prednisolone, but a difference was seen for anti-Ro-60 (p<0.05). CONCLUSIONS: Prednisolone is a potent anti-inflammatory and immunosuppressive drug commonly used in autoimmune diseases. Our study shows that oral steroids are associated with reduced levels of several pro-inflammatory cytokines, but increased levels of pSS specific autoantibodies. The association between steroid use and increased antibody levels is not readily explained by known steroid effects, and should therefore be confirmed in further studies. Lower levels of pro-inflammatory cytokines indicate a beneficial effect of oral steroids in this patient group.