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ID PMID Title PublicationDate abstract
17584326 Paclitaxel-induced cutaneous lupus erythematosus in patients with serum anti-SSA/Ro antibo 2007 Jul Two women (aged 46 and 56 years) developed annular erythema on their sun-exposed skin, including their forearms and faces, after biweekly i.v. administration of paclitaxel for 3-4 months to treat breast cancer. Both cases showed interface changes of basal keratinocytes and high titers of serum anti-SSA/Ro antibody. The diagnoses of paclitaxel-induced cutaneous lupus erythematosus (LE) were made because the skin eruptions cleared rapidly within 3 weeks after the paclitaxel treatment was discontinued. Paclitaxel is proposed to be a causative agent that can provoke cutaneous LE.
17576563 Diffuse disseminated candidiasis in a patient with Felty's syndrome: a case report. 2007 Nov Severe granulocytopenia predispose patients with Felty's syndrome to severe infectious diseases. The following report deals with an occurrence of chronic disseminated candidiasis in a patient with Felty's syndrome who presented with prolonged and severe granulocytopenia. To the best of our knowledge this coexistence has never been described before.
16287920 Clinical significance of quantitative immunohistology in labial salivary glands for diagno 2006 Apr OBJECTIVES: Because patients with primary Sjögren's syndrome (pSS) are at risk of developing other autoimmune phenomena and malignant lymphoma, it is important to distinguish pSS from non-Sjögren's (nSS) sicca syndrome. However, this distinction might be difficult because of the lack of a gold standard for pSS. We studied the clinical significance of quantitative immunohistology (QIH) in labial salivary glands for diagnosing pSS. METHODS: In a model mimicking the making of a clinical diagnosis, five experts diagnosed 396 patients as nSS, 'indefinite', pSS or secondary SS (sSS) using 25 clinical parameters. Patients were diagnosed twice, namely without (yielding gold-standard diagnoses) and with knowledge of QIH. The numbers of changes in diagnosis from 'indefinite' to 'definite' (nSS, pSS or sSS) or vice versa were compared. Patient groups with vs without a changed diagnosis in the four gold-standard diagnosis groups were compared regarding objective autoimmune parameters. RESULTS: Sensitivity, specificity, positive and negative predictive value for abnormal QIH in pSS vs nSS were 93, 86, 76 and 96%, respectively. Changes in diagnosis from 'indefinite' to 'definite' (31%) were found more often (P = 0.00) than changes from 'definite' to 'indefinite' (10%). Knowledge of QIH distinguished patient groups within the gold-standard nSS, indefinite and pSS patient group with regard to autoimmune parameters. CONCLUSION: In view of the consequences of distinguishing pSS from nSS, these results point to an additional diagnostic role for QIH in clinical practice.
17006270 Epstein-Barr virus associated Hodgkin lymphoma in a 9-year-old girl receiving long-term me 2006 Sep We describe a case of Hodgkin lymphoma developing in a 9-year-old girl with polyarticular, rheumatoid factor-positive juvenile idiopathic arthritis treated with methotrexate (MTX), prednisone, and naproxen for 5 years. Pathologic and molecular analyses revealed that the Hodgkin cells contained Epstein-Barr virus and the viral DNA was monoclonal. She achieved complete remission after MTX withdrawal, chemotherapy, and radiation. To the best of my knowledge, this is the sixth report of Hodgkin lymphoma in patients with juvenile idiopathic arthritis receiving low dose MTX therapy.
19008012 Adult onset Still's disease after first cycle of pemetrexed and gemcitabine for non-small 2009 Apr Pemetrexed is a multitargeted antifolate approved for the second-line treatment of locally advanced or metastatic non-small cell lung cancer. The combination of pemetrexed with gemcitabine has been studied in several clinical trials showing a promising antitumor activity with a mild toxicity profile. We present the case of a patient who experienced fever, arthralgia, skin rash and high serum ferritin levels after first cycle of this chemotherapy combination, compatible with an adult onset Still's disease. This adverse event has not been previously reported.
18318250 [Case of HLA-DR8 positive sarcoidosis following polymyositis and Sjögren's syndrome]. 2008 Feb A 69-year-old woman had been found to have idiopathic interstitial pneumonia (fibrotic NSIP) in 1997. Proximal muscle weakness appeared in April 2005. Chest CT revealed hilar and mediastinal lymphadenopathy. Polymyositis and Sjögren's syndrome were subsequently diagnosed. We assumed that the interstitial pneumonia had preceded polymyositis and Sjögren's syndrome. A muscle biopsy and transbronchial needle aspiration biopsy demonstrated noncaseating epithelioid cell granulomas. A diagnosis of sarcoidosis complicated with polymyositis and Sjögren's syndrome was made from these findings. Moreover, her HLA genotype contained DR8. HLA-DR8 is considered to be associated with polymyositis, Sjögren's syndrome, and sarcoidosis in Japanese patients. This case suggests the possibility that there are common immunological and genetical pathogenetic mechanisms in autoimmune diseases and sarcoidosis.
17691581 [Effect of electroacununcture on sex hormone levels in patients with Sjögren's syndrome]. 2007 OBJECTIVE: To observe the effect of electroacupuncture (EA) on serum testosterone (T), estradiol (F2), luteotropic hormone (LH), follicle-stimulating hormone (FSH), progesterone (P), and prolactin (PRL) in patients with Sjögren's Syndrome (SS) in order to analyze the correlation between the adjustment effect of EA and changes of hormone levels. METHODS: Fifty-seven middle-aged and old women with SS were divided into medication group (n=27) and acupuncture group (n=30). Patients in acupuncture group were treated with EA (80 Hz, 2.5 mA) of Shenshu (BL 23), Taixi (KI 3), Hegu (LI 4), etc. for 30 min, once every other day, and those of medication group were treated with oral administration of hydroxyl-chloroquine (6 mg/kg per day), oral transfer agent liquid, Vit B1, Vit B2, Vit C, fish-liver oil, one tablet/time, t.i.d., and pilocarpine (20 mg/d), continuously for 2 months. Venous blood samples were collected before and after the treatment to examine contents of the abovementioned sex hormones by using electro-chemiluminescent immunoassay (CLIA). RESULTS: (1) Before the treatment, no significant differences were found between two groups in the levels of serum T, E2, LH, FSH, P and PRL (P > 0.05). After the treatment, self-comparison of each group showed that serum EF and T contents in acupuncture group increased significantly (P < 0.01) and serum LH content decreased significantly (P < 0.01); while in medication group, only serum LH decreased markedly (P < 0.05) in comparison with its basic value of pretreatment. No significant changes were found in serum P, FSH and PRL levels in both groups after the treatment (P > 0.05). The results suggested that the therapeutic effect of acupuncture group was better than that of medication group in regulating multiple sex hormones. CONCLUSION: EA can effectively adjust E2, T and LH levels in Sjögren's Syndrome patients and improve most patients' clinical symptoms, and the therapeutic effect of EA is better than that of medication.
17318272 Quantitative salivary gland scintigraphy can distinguish patients with primary Sjøgren's 2007 Nov Abnormal findings on salivary gland scintigraphy (SGS) are part of the classification criteria for Sjøgren's syndrome (SS), but SGS is operator dependent and poorly standardised. We studied the use of quantitative data on the uptake, concentration and excretion of the four major salivary glands in the evaluation of sicca patients. During an initial clinical evaluation for sicca symptoms (mean duration, 51 months), 24 subjects were classified as either SS (n = 8) or isolated sicca (IS; n = 16). SGS was then performed after i.v. injection of 200 MBq pertecnetat. Digitalised quantitative data on time-to-peak uptake (Tmax), peak tracer distribution (C%) and stimulated excretion (E%) were calculated from time-activity curves and compared between groups and controls (n = 8) and correlated to clinical data. Statistical analysis was performed with non-parametric tests. SS patients had longer Tmax in both parotic glands (18.1 min; p < 0.01)) and both submandibular glands (mean 13.7 min, p < 0.05); whereas Tmax in IS patients was similar as in controls in both parotic (10.4 min; p > 0.2) and submandibular glands (9.4 min; p > 0.4). C% was significantly lower in the parotic glands of both the SS and the IS group compared to the controls (p < 0.01). E% was significantly reduced in SS patients (16.3% for parotic and 17.4% for submandibular glands; p < 0.01); whereas in the IS patients, excretion (32, 2% for parotic and 26, 9% for submandibular glands) was similar from all glands as in the control groups (35, 2% for parotic and 27, 8% for submandibular glands). No correlation was found between these SGS results and age, focus score, erythrocyte sedimentation rate, serum creatinin or immunoglobulin levels. No IS patient progressed to full-blown pSS during the 4 years of follow-up. Quantitative SGS data are useful and objective tools to distinguish patients with SS.
18397708 Hypokalemic quadriparesis associated with renal tubular acidosis in a patient with Sjögre 2008 Apr Sjögren's syndrome is an autoimmune exocrinopathy that involves both glandular and extra-glandular systems. We report a 25-year-old woman who had rapidly progressive quadriparesis. Biochemical investigations showed severe hypokalemia with hyperchloremic metabolic acidosis diagnosed as distal renal tubular acidosis. Salivary gland biopsy revealed Sjögren's syndrome as the underlying cause. She recovered following from quadriparesis potassium and alkali replacement.
18542976 Outcome after temporary physeal stapling for knee valgus deformity in children with JIA. 2008 Oct INTRODUCTION: Majority of children with secondary knee valgus deformity due to juvenile idiopathic arthritis (JIA) are affected by the polyarthritic disease subtype. Progressive rheumatoid knee destruction in patients with JIA and valgus deformity may necessitate total knee replacement (TKR) at a young age. Temporary physeal arrest is a safe and effective method for correction of knee valgus malalignment prior to closure of the epiphyseal growth plates even during active arthritis. RESULTS: Most of the angular correction achieved in the stapled knees (n = 103) in the present cohort remained the same through the long-term follow-up though in some patients the deformity did recur. The advantages of angular correction prior to possible future TKR include easier soft tissue balancing, diminished bony deformity and less deranged collateral ligaments reducing the need for expensive custom and constrained implants. CONCLUSION: However, the effect of the correction on postponing the early need for TKR is limited.
16642931 [Chronic inflammatory demyelinating polyneuropathy followed by systemic lupus erythematosu 2006 Mar We report a 60-year-old man with chronic inflammatory demyelinating polyneuropathy (CIDP) accompanying systemic lupus erythematosus (SLE) and Sjögren syndrome (SS). He was admitted to our hospital because of progressive weakness and dysesthesia in the distal parts of the bilateral upper and lower extremities. We diagnosed the illness as CIDP and treated him with steroids, plasma filtration and high-dose intravenous immunoglobulin therapy (IvIg). Consequently, his symptoms improved gradually and he was discharged. However, 2 years after the first admission, he experienced dyspnea on effort and chest X-ray demonstrated right pleural effusion. Consequently, he gradually developed gait disturbance, loss of taste, and severe dysesthesia in his lower limbs. Therefore, he was admitted again. On neurological examination, mild weakness was detected in all limbs distally. Deep tendon reflexes were absent. The sensation of position and vibration was diminished in the fingers and toes. He could not walk by himself and demonstrated an ataxic gait with a wide base. Examination of cranial nerves demonstrated no abnormalities, except for loss of taste. Serological examination demonstrated positive auto-antibodies including antinuclear, anti-DNA, and anti-SS-A antibodies. Urinalyses showed albuminuria and microscopic hematuria. Cerebrospinal fluid (CSF) was clear and colorless, containing 3 mononuclear cells per cubic mm, with a protein of 275 mg/dl. Magnetic resonance images (MRIs) of the brain and entire spine were normal. Neurophysiological studies demonstrated an absence of sensory nerve action potentials in the right arm and markedly slow conduction velocities, conduction block in the ulnar forearm segment and absence of F waves in all limbs. The renal biopsy revealed lupus nephritis. The lip biopsy demonstrated chronic sialoadenitis consistent with SS, altough patient did not show symptoms of arthritis or vasculitis. It is well known that mononeuritis multiplex and acute demyelinating polyneuropathy accompany SLE. However there are few reports that describe the occurrence of CIDP in patients with SLE, and the association of "CIDP-like" neuropathy in patients with SS. Our case suggests that the autoimmune disease associated with SLE and SS may induce "CIDP-like" inflammatory demyelinating polyneuropathy.
16622725 Hypokalemic paralysis and osteomalacia secondary to renal tubular acidosis in a case with 2006 A 39-year-old Japanese woman was admitted to our hospital for severe weakness owing to potassium deficiency caused by type 1 renal tubular acidosis (RTA1). Sicca complex, serological tests, and lip biopsy revealed that she had Sjögren's syndrome (SS). Acidosis was corrected by alkali supplement treatment. She also had an impaired renal function with proteinuria, and high absorbance on Ga scintigram was recognized in both kidneys. She was taking warfarin potassium after aortic valve substitution due to aortic regurgitation, therefore renal biopsy was not performed. Prednisone (20 mg/day) was administered for renal inflammation. One month later, she suffered severe chest wall pains with some local tender points over the costae of both sides, which was presumed to be due to pseudo-fractures based on osteomalacia. Hypokalemic paralysis and osteomalacia should be taken into consideration in the diagnosis of SS with RTA1.
18565301 [Biological treatment of rare inflammatory rheumatic diseases]. 2008 Jun 9 The current status of the use of biological medicine in the treatment of adult onset morbus still, Wegeners granulomatosis and systemic lupus erythematosus (SLE) is reviewed. The need for controlled trials is emphasized. Anti-CD20 treatment for SLE patients with kidney involvement and patients with Wegeners granulomatosis seems promising. Anti-TNF and IL1 receptor antagonist can control disease activity in most patients with adult morbus still.
18493766 A case of mixed connective tissue disease complicated with hypertrophic obstructive cardio 2008 Oct A 54-year-old female was diagnosed as mixed connective tissue disease (MCTD) complicated with secondary Sjögren's syndrome. Although she had no dyspnea on exertion, the chest X-ray showed cardiomegaly with interstitial pneumonia. The echocardiogram demonstrated asymmetric hypertrophy of the interventricular septum. Diagnosis of hypertrophic obstructive cardiomyopathy (HOCM) was confirmed by left ventriculography and myocardial biopsy. She was treated with prednisolone, resulting in improvement of swollen hand, elevated muscle enzymes and interstitial pneumonia. A rare complication of HOCM with MCTD was described.
18183612 Retinal microangiopathy and rapidly fatal cerebral edema in a patient with adult-onset Sti 2008 May Hemophagocytic lymphohistiocytosis (HLH) is a complex inflammatory disease with multiple diagnostic and therapeutic pitfalls. The congenital form, referred to as familial hemophagocytic lymphohistiocytosis (FHL), is often associated with cerebromeningeal involvement, whereas neurological complications are not characteristic of the adult form of secondary HLH (sHLH). Here we report the case of a 20-year-old woman with adult-onset Still's disease (AOSD), retinal microangiopathy and concurrent macrophage activation syndrome (MAS), in the context of sHLH. Following treatment with etanercept, ibuprofen, methylprednisolone, and phenylbutazone for 3 weeks, MAS deteriorated and fatal cerebral edema occurred within only 24 h. The clinical signs and neuropathological findings are discussed with special emphasis on possible relationships between the aggravation of MAS and therapeutic interventions for AOSD. In conclusion, even the slightest sign of mental decline in a patient with AOSD must be considered central nervous system MAS which can be rapidly fatal.
19115058 Cloak and dagger: the case for adult onset still disease and hemophagocytic lymphohistiocy 2009 Jun Adult onset Still's disease (AOSD) is a rare systemic inflammatory disorder of unknown etiology. Systemic onset juvenile idiopathic arthritis (SoJIA) is the preferred nomenclature of Still's disease. Strong association with so-called macrophage activation syndrome (MAS) may provide a clue to the understanding of the distinctive pathogenetic features of SoJIA. MAS is a severe, potentially life-threatening complication characterized by the excessive activation of well-differentiated macrophages. It is more appropriately named autoimmune disease associated reactive hemophagocytic lymphohistiocytosis (ReHLH), a subset of a histiocytic disorder: class II histiocytosis hemophagocytic lymphohistiocytosis (HLH). The relation of SoJIA with HLH is still under debate. We propose that MAS, HLH, SoJIA, and AOSD are indeed the same disease, in different clinical presentations that may be classified based on severity and laboratory findings, but with essentially the same physiopathogenesis. We propose that the case described by Hong & Lee (Rheumatol Int 2008) was actually an AOSD-associated MAS/RHS/ReHLH fulminant disease.
18677223 Antibody protein array analysis of the tear film cytokines. 2008 Aug PURPOSE: Many bioactive proteins including cytokines are reported to increase in dry eye disease although the specific profile and concentration of inflammatory mediators varies considerably from study to study. In part, this variability results from inherent difficulties in quantifying low abundance proteins in a limited sample volume using relatively low sensitivity dot ELISA methods. Additional complexity comes with the use of pooled samples collected using a variety of techniques and intrinsic variation in the diurnal pattern of individual tear proteins. The current study describes a recent advance in the area of proteomics that has allowed the identification of dozens of low abundance proteins in human tear samples. METHODS: Commercially available stationary phase antibody protein arrays were adapted to improve suitability for use in small volume biological fluid analysis with particular emphasis on tear film proteomics. Arrays were adapted to allow simultaneous screening for a panel of inflammatory cytokines in low volume tear samples collected from individual eyes. RESULTS: A preliminary study comparing tear array results in a small population of Sjögren's syndrome patients was conducted. The multiplex microplate array assays of cytokines in tear fluid present an unanticipated challenge due to the unique nature of tear fluid. The presence of factors that exhibit an affinity for plastic, capture antibodies and IgG and create a complex series of matrix effects profoundly impacting the reliability of dot ELISA, including with elevated levels of background reactivity and reduction in capacity to bind targeted protein. CONCLUSIONS: Preliminary results using tears collected from patients with Sjögren's syndrome reveal methodological advantages of protein array technology and support the concept that autoimmune-mediated dry eye disease has an inflammatory component. They also emphasize the inherent difficulties one can face when interpreting the results of micro-well arrays that result from blooming effects, matrix effects, image saturation and cross-talk between capture and probe antibodies that can greatly reduce signal-to-noise and limit the ability to obtain meaningful results.
18355394 Headache in primary Sjögren's syndrome: a prevalence study. 2008 Sep OBJECTIVES: To determine the prevalence of headache in patients with primary Sjögren's syndrome (pSS) and to examine the relationship between headache types and clinical, serologic features of the disease. METHODS: The study enclosed 133 patients with the diagnoses of pSS and 97 healthy controls. A questionnaire designed to assess the presence of headache and if present to classify it according to the criteria of the International Headache Society was used. RESULTS: In 133 of the pSS patients evaluated, 104 had headache. No association was present between types of headache and the clinical and laboratory manifestations of the disease. Both migraine and tension-type headache were more common in patients with pSS when compared with healthy controls (P < 0.001). CONCLUSIONS: The high prevalence of migraine in pSS patients might be explained by a vascular headache triggered by immuno-mediated disease activity without an obvious clinic or laboratory marker.
17637529 Sarcoidosis (Heerfordt syndrome): a case report. 2007 We report the case of a 22-year-old woman who is suspected of having primary Sjögren s syndrome. She complaining of bilateral swelling of eyelids and the parotid glands of three weeks duration. Physical examination revealed a bilateral enlargement of both parotid glands, which were solid and painful. Sjögren s syndrome was suspected at that stage, and the serologic and specific analysis were done. All these tests didn t find any autoimmune or visceral features typical of Sjögren s syndrome and autoantibodies were negative. During follow-up time the right facial nerve palsy developed. Pulmonary radiography revealed bihilar lymphadenopathy and labial salivary gland biopsy revealed non-caseating granuloma. The patient was classified as having stage I sarcoidosis. This case demonstrates the importance of being aware of the leading clinical signs and symptoms in case of Heerfordt syndrome.
16467042 Epidemiology and outcome of adult-onset Still's disease in Northern Norway. 2006 Jan BACKGROUND: Adult-onset Still's disease (AOSD) is considered a rare disease, but few data exist on the incidence and prevalence of AOSD. This study has analysed the epidemiology, disease presentation, and outcome of AOSD in a stable homogeneous population in Northern Norway. METHODS: A retrospective cohort study of all AOSD patients registered in 1990-2000 at the only hospital in the region with a Rheumatology Service. Clinical diagnosis and exclusion of patients were directed by the Yamaguchi criteria for AOSD. Demographic and clinical data at baseline were extracted from patient records and supplemented with data gathered at control visits. Data were analysed with nonparametric methods. RESULTS: AOSD was ascertained in 13 patients; the estimated annual AOSD incidence was 0.4/100,000 adults (95% CI 0.11-0.97), while point prevalence of AOSD increased from 3.4/100,000 (95% CI 0.8-9.4) in 1990 to 6.9/100,000 in 2000 (95% CI 2.7-14.2). Mean diagnostic delay was 5.2 months (range 0.5-18). Serum ferritin > 5 times the normal upper level had 63% diagnostic sensitivity. During 69 months' follow-up, one patient died, 6/13 patients achieved sustained remission, while six patients developed a chronic progressive (n = 3) or a relapsing/remitting disease course (n = 3). Four of these six patents had to enter social security programmes. CONCLUSION: The annual incidence of AOSD in Northern Norway is at least 0.4/100,000 adults. AOSD in this region is more prevalent than in France or Japan, affects more males, and approximates to the prevalence of juvenile Still's disease. Half of all patients have a monocyclic disease course, while mortality and invalidity occur in patients with chronic disease.