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ID PMID Title PublicationDate abstract
19746766 Primary cutaneous nodular amyloidosis: case report and review of the literature. 2009 Aug Primary cutaneous nodular amyloidosis (PCNA) is a rare form of primary cutaneous amyloidosis. It presents as waxy yellow-red nodules that are located preferentially on the lower extremities, face, scalp, and genitals. Recognition of this condition is of particular importance, as primary systemic amyloidosis can have a similar cutaneous presentation. We report a case of PCNA in a 52-year-old woman with systemic lupus erythematosus (SLE) and Sjögren syndrome (SS). We discuss the need to evaluate for systemic disease and provide a concise review of the literature focusing on clinical presentation, disease associations, and management.
19375051 [Association between thymic MALT lymphoma and Sjögren's syndrome]. 2009 Apr INTRODUCTION: Thymic mucosa-associated lymphoid tissue (MALT) lymphoma is a rare pathology, often associated with autoimmune diseases. The authors report the case of an Asian woman with Sjögren's syndrome. OBSERVATION: A 48-year-old Chinese woman, without past medical history and a non-smoker, presented an alteration in her overall condition, dyspnoea at exercise, inflammatory polyarthralgia, and a dry eye and mouth syndrome over the last few months. Thoracic tomodensitometry detected an anterior heterogenic cystic mediastinal mass. A mediastinotomy was performed. The diagnosis of the surgical biopsy was thymic MALT lymphoma. The authors also diagnosed Sjögren's syndrome with the presence of four diagnostic criteria. Chemotherapy by rituximab, cyclophosphamide, vincristine, prednisone induced major tumoral regression. The patient declined surgery and will be monitored. CONCLUSION: Thymic MALT lymphoma is a rare pathology. There is a high correlation with autoimmune diseases, like Sjögren's syndrome. Its appearance is that of an anterior mediastinal mass with a cystic component. The treatment is not well codified and is most often based on surgical resection, eventually followed by chemotherapy or radiotherapy. As far as the authors know, this is the second case of thymic MALT lymphoma treated by exclusive chemotherapy.
19231036 [Sarcomatoid carcinoma in a patient with Sjögren's syndrome]. 2009 Mar Sarcomatoid carcinoma is an extremely rare small bowel tumor whose clinical manifestations are insidious and nonspecific, ranging from diffuse abdominal pain to gastrointestinal bleeding or intestinal occlusion. Thus, diagnostic delay is highly common with poor treatment outcome and prognosis. To date, only 20 cases have been reported in the literature. We describe the case of a small bowel sarcomatoid carcinoma localized in the jejunum, with emphasis on the clinical and pathological features of this entity. The hypothetical association with Sjögren's syndrome, an autoimmune disease, is also discussed.
19213588 Multicentric Castleman's disease mimicking adult-onset Still's disease. 2009 May Castleman's disease is a rare lymphoproliferative disorder having two types of presentation: the localized and the multicentric form. Multicentric Castleman's disease (MCD) typically presents with constitutional symptoms, generalized peripheral lymphadenopathy, hepatosplenomegaly, and laboratory markers of inflammation. Rash and arthritis may also be initial complaints of this disease. In these cases, MCD can resemble adult-onset Still's disease (AOSD), especially if the arthritis precedes other manifestations. We describe a patient with initial clinical suspicion of AOSD. Eighteen months later evidence of MCD was ascertained when the patient developed insidiously growing axillary lymphadenopathies. Despite its rarity, MCD should be borne in mind in the differential diagnosis of patients with suspicion of AOSD.
19198237 [Pulmonary hypertension in a patient on chronic hemodialysis]. 2009 Jan A 52-year-old woman with end-stage renal disease and long-term hemodialysis complained of worsening exertional dyspnea. A chest X-ray showed cardiomegaly. She was admitted to our hospital because an echocardiogram suggested pulmonary hypertension. Right heart catheterization revealed pulmonary hypertension, but pulmonary perfusion scintigraphy with Tc-99m-MAA showed no evidence of pulmonary thromboembolism. We gave her a diagnosis of pulmonary arterial hypertension associated with Sjögren syndrome on the basis of a positive serological test (SS-A. SS-B) and the findings of lip biopsy. After four months of therapy with bosentan, her 6-minute walk distance, estimated pulmonary arterial pressure and brain natriuretic peptide (BNP) improved. Bosentan is mainly cleared by hepatic elimination and its dialysis clearance is low. Bosentan for the treatment of pulmonary hypertension was safe as well as effective in this patient with end-stage renal disease and hemodialysis. In consideration of the relationship between pulmonary hypertension and end-stage renal disease, and hemodialysis, bosentan was considered to be a reasonable and effective treatment.
18757116 [Orofacial manifestations of systemic sclerosis: a study of 30 consecutive patients]. 2009 Jan INTRODUCTION: The face is frequently involved in systemic sclerosis. The main stomatologic manifestations include limited mouth opening, xerostomia, skin atrophy, trigeminal neuralgia. The objective of this study was to describe oral and facial manifestations observed in scleroderma patients from our cohort. METHODS: Between March and October 2006, a stomatologic consultation was included in the follow-up of scleroderma patients seen during consultation or daily hospital in internal medicine or dermatology units. Demographic, clinical and biological data were collected. Stomatologic examination comprised measure of the mouth opening, sugar's and Schirmer's tests, orthopantomogram analysis, and evaluation of the repercussion of symptoms on quality of life using a visual analogical scale (VAS between 0 and 10). RESULTS: This study included 30 patients (women 87%, mean age 58.6+/-13.6 years). Mean duration of systemic sclerosis (n=20 limited cutaneous form, n=10 diffuse form) was eight years. Stomatologic manifestations were: skin atrophy (n=28), peribuccal rhagades (n=25), telangiectasia (n=21), decreased mouth opening (n=20), xerostomia (n=20), xerophtalmia (n=16), periodontal ligament space widening (n=10), bone resorptions (n=2), trigeminal neuralgia (n=1). Xerostomia was considered more discomforting (mean VAS=3.8) than decreased mouth opening (mean VAS=2.6). Xerostomia was the second more discomforting sign of scleroderma and was significantly associated to the limited cutaneous form (p=0.045) and to anticentromeres antibodies expression (p=0.002). Decreased mouth opening was correlated to oesophageal involvement (p=0.025). CONCLUSION: Oral and facial manifestations are frequently observed in scleroderma patients. These manifestations lead to major functional discomfort, mainly due to decreased mouth opening that seems to be frequently associated to oesophageal involvement. Xerostomia is also frequent and is commonly observed in anticentromere antibodies positive cutaneous limited forms of systemic sclerosis. Evolution of radiographic abnormalities like periodontal ligament space widening (33% of cases), or osteolytic lesions (7%) is poorly known.
20844306 [Differential diagnosis of neuromyelitis optica spectrum disorders]. 2010 Sep Neuromyelitis optica (NMO) is a demyelinating disease of the central nervous system that preferentially affects the optic nerves and spinal cord. NMO-IgG/anti-aquaporin 4 antibody (AQP4-Ab) is considered as a specific diagnostic marker for NMO. A previous study using animal models passively transferred with AQP4-Ab has partially proven that NMO-IgG/AQP4-Ab has an effector function in the pathogenesis of NMO, exemplifying the diagnostic significance of this antibody. Further, this marker can be used to differentiate the limited forms of NMO, such as recurrent myelitis or optic neuritis or NMO with isolated cerebral/brainstem lesions during the early course of the disease, from other diseases with a different etiology. NMO spectrum disorders (NMOSD) comprise these clinically heterogeneous conditions, all of which are positive for AQP4-Ab. However, few patients show clinical characteristic features of NMO, without AQP4-Ab positivity. We should be careful to introduce interferon beta for the prevention of relapse to these seronegative but suspicious for NMO patients. A few NMOSD patients have also been diagnosed with systemic lupus erythematosus (SLE) or Sjogren syndrome (SjS). However, there have been no reported cases of patients with SLE/SjS who do not exhibit any neurological symptoms and AQP4-Ab-positivity, and it is likely that these 2 autoimmune diseases incidentally overlap. NMO might follow myasthenia gravis (MG), after thymectomy for the treatment of MG. Taken together,as in the case of other systemic autoimmune diseases,an antibody-mediated pathomechanism of NMO is suggested.
20661405 Autoimmune hepatitis in patients with primary Sjögren's syndrome: a series of two-hundred 2010 Mar 25 Based on the revised criteria of the American-European Consensus Group, we retrospectively established the diagnosis of primary or secondary Sjögren's syndrome for 202 patients referred to a Sjögren's syndrome clinic. Of these, 58 patients and 8 patients fulfilled criteria for primary and secondary Sjögren's syndrome, respectively. Of the 58 patients with primary Sjögren's syndrome, one (1.7%) had definite autoimmune hepatitis, as defined by the International Autoimmune Hepatitis Group diagnostic criteria. One additional symptomatic patient who did not fulfill criteria for primary Sjögren's syndrome had definite autoimmune hepatitis. None of the patients with secondary Sjögren's syndrome had definite autoimmune hepatitis. Two (1%) of the 194 patients with primary Sjögren's syndrome or clinical symptoms had primary biliary cirrhosis. These values are lower than those reported by prior studies with smaller patient populations and likely represent a more accurate estimate of the true prevalence of these diseases in patients with primary Sjögren's syndrome.
19938253 Laser as a therapy for dry mouth symptoms in a patient with Sjögren's syndrome: a case re 2009 May This clinical case study reports on dry mouth symptoms in a patient with Sjögren's syndrome (SS) who was treated with laser phototherapy (LPT). A 60-year-old woman diagnosed with SS was referred to the laboratory for lasers in dentistry to treat her severe xerostomia. A diode laser (780 nm, 3.8 J/cm2, 15 mW) was used to irradiate the parotid, submandibular, and sublingual glands, three times per week, for a period of 8 months. The salivary flow rate and xerostomia symptoms were measured before, during, and after LPT. Dry mouth symptoms improved during LPT. After LPT, the parotid salivary gland pain and swelling were no longer present. Treatment with LPT was an effective method to improve the quality of life of this patient with SS.
20724508 Prepatellar bursitis due to Brucella abortus: case report and analysis of the local immune 2010 Dec A case of prepatellar bursitis in a man with chronic brucellosis is presented. Brucella abortus biotype 1 was isolated from the abundant yellowish fluid obtained from the bursa. Clinical and epidemiological data did not suggest a direct inoculation of the agent in the bursa. However, the patient mentioned occasional local trauma due to recreational sports, which may have constituted a predisposing factor. As determined by ELISA, there were higher levels of IgG against Brucella LPS and cytosolic proteins detected in the patient's bursal synovial fluid when compared with serum. Levels of proinflammatory cytokines (tumour necrosis factor alpha, interleukin 1 beta, gamma interferon, interleukin 8 and MCP-1) were higher than in synovial fluids obtained from patients with rheumatoid arthritis and a patient with septic arthritis, and a zymographic analysis revealed a gelatinase of about 92 kDa. These findings indicate that it may be possible to diagnose brucellar bursitis by measuring specific antibodies in the bursal synovial fluid. In addition, our findings suggest a role of increased local levels of proinflammatory cytokines and gelatinases in the inflammatory manifestations of brucellar bursitis.
20167120 Interleukin-17A upregulates receptor activator of NF-kappaB on osteoclast precursors. 2010 INTRODUCTION: The interaction between the immune and skeletal systems is evidenced by the bone loss observed in autoimmune diseases such as rheumatoid arthritis. In this paper we describe a new mechanism by which the immune cytokine IL-17A directly affects osteoclastogenesis. METHODS: Human CD14+ cells were isolated from healthy donors, cultured on dentine slices and coverslips and stimulated with IL-17A and/or receptor activator of NF-kappaB ligand (RANKL). Osteoclast differentiation was evaluated by gene expression, flow cytometry, tartrate-resistant acid phosphatase staining, fluorescence and electron microscopy. Physiologic bone remodelling was studied in wild-type (Wt) and IL-17A-/- mice using micro-computer tomography and serum RANKL/osteoprotegerin concentration. Functional osteoclastogenesis assays were performed using bone marrow macrophages isolated from IL-17A-/- and Wt mice. RESULTS: IL-17A upregulates the receptor activator for NF-kappaB receptor on human osteoclast precursors in vitro, leading to increased sensitivity to RANKL signalling, osteoclast differentiation and bone loss. IL-17A-/- mice have physiological bone homeostasis indistinguishable from Wt mice, and bone marrow macrophages isolated from these mice develop fully functional normal osteoclasts. CONCLUSIONS: Collectively our data demonstrate anti-IL-17A treatment as a selective therapeutic target for bone loss associated with autoimmune diseases.
19749141 Inflammatory bowel disease and lymphoproliferative disorders: the dust is starting to sett 2009 Oct The risk of lymphoproliferative disorders (LDs) has become a major concern for clinicians managing patients with inflammatory bowel disease (IBD). Yet it is difficult to distinguish the possible responsibility of immunosuppressive therapy from the background risk due to the inflammatory disorder itself. LDs are clonal B or T cell proliferation showing considerable heterogeneity and the incidence has increased since the 1970s. The strongest and best-established risk factors for LDs are primary and acquired immunodeficiency (HIV, immunosuppressant), notably via defective immune surveillance of Epstein-Barr virus. In many auto-immune diseases (eg, Sjögren's syndrome), inflammatory diseases (eg, rheumatoid arthritis) or chronic suppuration (chronic pyothorax), the risk of LD is increased. In IBD patients, in general, the risk of LD seems to be similar to or very slightly higher than in the general population. The role of immunosuppressants in lymphomagenesis is difficult to individualise because other factors potentially involved are inter-linked. Concordant data suggest that thiopurine therapy is associated with a moderately increased risk of LD. Data regarding methotrexate are scarce and come from diseases other than IBD but the risk seems low. Data regarding risk of LD in IBD patients receiving anti-tumour necrosis factor alpha (TNFalpha) agents are insufficient at this time, mainly because most of the patients are co-treated with thiopurines. The recently individualised risks of hepatosplenic T cell lymphoma and fatal post-mononucleosis LD, in young male patients with IBD who are co-treated with anti-TNFalpha and thiopurines, and EBV-seronegative IBD males, respectively, are probably low but remain to be better quantified.
20680378 Clinical findings in parvovirus B19 infection in 30 adult patients in Kyoto. 2011 Feb To relate the clinical findings of parvovirus B19 infection to the phase of the disease, we performed a retrospective chart review of 30 adult patients who tested positive for IgM antibody against parvovirus B19 at our hospital from March 2003 to November 2008. Median patient age was 38 years, with 86.7% aged between 26 and 45 years. The male-to-female ratio was 4:26 (86.7% female). Symptoms in the first phase were mainly flu-like, including fever, headache, or myalgia. Symptoms in the second phase were arthralgia in 24 (85.7%) and rash in 23 (82.1%). Fever was observed in 21 (70.0%), and 22 (75.9%) were found to be lymphopenic. The onsets in 73.3% of cases were concentrated within 10.1% of the study period, an observation nearly consistent with an outbreak of erythema infectiosum. Three patients had symmetrical swelling of joints, all of whom also had rash. Most patients visited the hospital within a week of onset and prognosis was favorable. In the parvovirus B19 infection, flu-like symptoms were frequent in the first phase, while rash and arthralgia were common in the second. Female sex, age between 26 and 45, and presence of rash, arthralgia, fever, and lymphopenia were clinical findings with a high frequency (≥70%), and these factors may contribute to diagnosis. In an era when early diagnosis and therapy is required in rheumatoid arthritis, it is important to recognize the parvovirus B19 infection with a presentation of acute arthritis and a favorable prognosis.
19319845 Evidence for the role of Th17 cell inhibition in the prevention of autoimmune diseases by 2009 Jan Deregulated production of interleukin-6 (IL-6) has been found in several chronic inflammatory autoimmune disorders, including rheumatoid arthritis (RA) and inflammatory bowel diseases. Treatment with tocilizumab, a humanized anti-human IL-6 receptor (IL-6R) antibody, significantly improved disease activity and inhibited the progression of joint destruction in RA patients, but the reason why IL-6 blockade causes improvement of RA is still unclear. In this review, we discuss the influence of anti-IL-6R antibody treatment on the differentiation of Th17 cells, which are thought to be involved in the pathogenesis of autoimmune diseases in animal models, present new results for the effect of anti-IL-6R antibody on the induction of Th17 cells in a mouse collagen-induced arthritis model, and come to the conclusion that anti-IL-6R antibody inhibited the differentiation of Th17 cells in mouse models. It is thought that this inhibitory action may contribute to the therapeutic effects of anti-IL-6R antibody in human autoimmune diseases.
19459572 [Recommendations for tuberculosis screening before and during treatment with tumour necros 2009 Mar Patients with an autoimmune disease, such as rheumatoid arthritis, ankylosing spondylitis, psoriatic arthritis, Crohn's disease, ulcerative colitis, uveitis or psoriasis, and treated with the anti-tumour necrosis factor (TNF) alpha inhibitors are at high risk of developing various infections including tuberculosis (TB). Serious infections are the result of the patients' immunocompromised status that is caused by the primary disease itself, as well as by previous immunosuppressive therapy. In order to decrease the risk of developing TB, prior to the introduction of the anti-TNF alpha therapy, all patients should undergo screening for TB. Experiences from the countries that have already implemented recommendations for TB screening show a significant decrease in TB occurrence in the anti-TNF alpha treated patients. The PPD skin test result is considered positive if in duration is of size > or =5 mm. The BCG vaccine applied at birth has no effect on interpretation of PPD test results in adults. The diagnosis of active TB is contraindicated for the introduction of the anti-TNF alpha therapy; first, such patients should receive the TB treatment; and 6 months after the completion of the TB treatment, the introduction of the anti-TNF alpha therapy may be considered. The patients with the diagnosis of the latent TB infection (LTBI) should not immediately start with the anti-TNF alpha therapy, but they should first receive the TB chemoprophylaxis; not earlier than a month upon the introduction of the TB chemoprophylaxis, the anti-TNF alpha therapy may be introduced. The first TB follow-up screening during the anti-TNF alpha therapy is recommended 6 months after the anti-TNF alpha therapy has been introduced and the next one should be scheduled after 12 months.
20135732 Radioscapholunate arthrodesis - a prospective study. 2009 The purpose of this prospective study was to evaluate pain levels, range of motion, patient activity and satisfaction after radioscapholunate (RSL) arthrodesis. This was in association with distal scaphoid excision and complete resection of the triquetrum. The non-union rate for radioscapholunate arthrodesis was examined and the results compared with previous studies. Twenty-three patients (14 males and nine females) with an average age of 47 (range 26-73) years underwent RSL fusion for post-traumatic osteoarthritis, rheumatoid arthritis and Kienböck's disease of the lunate with a mean follow-up of 32 (range 13-70) months. The absolute prerequisite for any of these groups of patients was a functional midcarpal joint which was assessed pre-operatively with radiographs and intra-operatively prior to RSL fusion. The average flexion to extension motion changed from 66 degrees to 57 degrees . The ulnoradial range of motion also increased to 43 degrees from a pre-operative value of 22 degrees . The patients visual analogue pain scores reduced from an average of 64 to 28 (p = 0.01). Nineteen patients had no restriction in activity and all but one was satisfied with the outcome. All patients remained in full time employment with ten returning to some form of sport. RSL fusion with excision of the distal pole of the scaphoid and the entire triquetrum led to minimal reduction in the flexion-extension arc of motion and an increase in the ulnoradial arc. There was also good pain relief and maintenance of a patient's function. Memory staples are also an effective method of securing fusion in the wrist obtaining similar results to that seen in forefoot surgery.
19962622 Complex measures and indices for clinical research compared with simple patient questionna 2009 Nov Indices of multiple measures have been developed to assess and monitor patients with rheumatic diseases, as no single "gold standard" measure is available for diagnosis, prognosis, and monitoring of all individual patients. Rheumatology indices generally include 4 types of measures from a standard medical evaluation: patient history, physical examination, laboratory tests, and imaging studies. Well-characterized indices are available for rheumatoid arthritis (RA), psoriatic arthritis, systemic lupus erythematosus (SLE), ankylosing spondylitis, vasculitis, osteoarthritis, fibromyalgia, and other rheumatic diseases. These indices are complex and applied widely in clinical research, but rarely are scored in usual rheumatology patient encounters, which generally are conducted without quantitative data other than laboratory tests. Information from a patient often is as prominent in clinical decisions as information from a physical examination or laboratory tests, and is easily collected as standardized "scientific" data on patient questionnaires designed for usual clinical care, which require minimal professional effort. Patient-derived data-along with physical examination, laboratory, and imaging data-are useful rheumatology "vital signs" to assess and monitor patient status, provide documentation, and improve the quality of clinical care, in addition to their possible value for clinical research. Differences between complex measures for research and simple questionnaires designed for usual clinical care might be more widely recognized, to promote quantitative measurement in the infrastructure of usual rheumatology care.
19483409 [New therapeutic strategy for autoimmune and chronic inflammatory disease based on clinica 2009 Jun Remarkable clinical effects were observed by IL-6 blockage with a humanized anti IL-6 receptor antibody in patients with Castleman's disease, rheumatoid arthritis, and juvenile inflammatory arthritis. This evidence suggests that the hyper-function of IL-6 is an essential key cytokine in the pathogenesis of the above diseases, in which many cytokines, chemokines, and inflammatory molecules are activated. We found, for example, TNF-alpha blocking therapy showed a reduction of acute phase proteins, such as CRP and SAA, however, the IL-6 blockade induced not only reduction but also normalization of CRP and SAA serum levels. To elucidate this in vivo phenomenon, we analyzed the expression of cytokine inducing CRP and SAA mRNA with the intracellular signal transduction mechanism in vitro. The results, indicated that the IL-6 signal was essential though the activation of STAT3 for the induction and augmentation of CRP or SAA by the associated stimulation with TNF-alpha or IL-1. Recently, it is now known that IL-6 is a regulatory molecule in the induction of Th17 cells with TGF-beta. Therefore, IL-6 blockage may potentially improve autoimmune diseases, beginning with the pathogenic initiation phase. We believe that unknown pathogenic inflammatory phenomena can be clarified using this analytical strategy and cytokine blocking therapy. Furthermore, in the future we hope to induce complete remission of autoimmune diseases by using cytokine blockage freely.
19349142 [Anti-TNFalpha therapy in systemic autoimmune and/or inflammatory diseases]. 2009 May TNFalpha plays a crucial role in the physiopathology of a large number of auto-immune and/or inflammatory systemic diseases. In addition to authorized indications including rheumatoid arthritis, ankylosing spondylitis, Crohn disease, ulcerative colitis, psoriatic arthritis and plaque psoriasis, TNFalpha blockers have been tested in a wide range of auto-immune and/or inflammatory diseases. TNFalpha blockers might be an option in refractory ANCA-associated vasculitis, sarcoïdosis, adult onset Still disease, Behçet disease, AA amyloïdosis and TRAPS. However, pertaining to the limited number of prospective randomized trails available, the small number of patients included and the poor methodology, it is difficult to define their place in the therapeutic strategy in these conditions. The therapeutic effect of TNFalpha blockers is often suspensive and disease flares are frequently observed during sustained treatment, as in the case of Behçet's disease. Published data do not support the use of TNFalpha blockers in connective tissue diseases.
21853899 [Clinical versus radiological mid-term results of total knee arthroplasty for degenerative 2010 Sep Total knee arthroplasty (TKA) is widely accepted method for treatment of severe osteoarthritis. The aim of this paper was to retrospectively review patients operated in our institution with total condylar knee arthroplasty due to osteoarthritis and assess clinical and radiological results of this procedure. All patients treated with TKA between 1998 and 2001 were reviewed, those with diagnosis of rheumatoid arthritis were excluded from the study. One hundred and one TKA in 68 patients were studied. WOMAC protocol and KSS (Knee Society Score) were used to evaluate patients clinically, and KSS alone for radiological analysis. Bone-implant interface has been studied, position of the implants and mechanical axis of the limb both pre- and postoperatively. Excellent and good results were achieved in 89% of TKA. Subjective self-assessment was usually worse than objective one. Radiolucency was found in 16 cases (more often around tibial component than the femoral one), usually without clinical symptoms of the loosening. An accurate alignment within the range of 3 to 9 degrees valgus has been found in 68% of the knees. Subjective scores were worse than objective clinical assessment. The clinical score was higher than radiological one. The tendency to varus tibial implant fixation was observed. Suboptimal implantation has not led to implant loosening in mid-term results.