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ID PMID Title PublicationDate abstract
15061688 Leflunomide for the treatment of rheumatoid arthritis in clinical practice: incidence and 2004 OBJECTIVE: Leflunomide is a novel disease modifying antirheumatic drug (DMARD). Because of reports on possible hepatotoxicity and adaptations in the recommendations for monitoring liver function during leflunomide treatment, we conducted a study to evaluate the incidence and severity of hepatotoxicity. METHODS: We included consecutive rheumatoid arthritis patients starting treatment with leflunomide in the region of Friesland (The Netherlands) between January 2000 and January 2002. During follow-up patient characteristics, disease characteristics, and clinical and laboratory data on liver functions were registered. Severity of hepatotoxicity was categorised using the National Cancer Institute Common Toxicity Criteria, as moderate (grade 2), severe (grade 3) or life threatening (grade 4). RESULTS: One hundred and one patients were followed for a median period of 10 months (range 0.5-12). Grade 2 or 3 elevations in any liver function blood test were recorded in a total of nine patients (8.9%). No grade 4 elevations were recorded. Four patients (4%) showed grade 2-3 aminotransferase elevations. Due to grade 2 hepatotoxicity one patient (1%) was withdrawn from leflunomide treatment, and one patient continued leflunomide at a reduced dose. In eight of nine patients with grade 2-3 liver function blood tests, these elevated liver function tests occurred within 6 months after starting leflunomide. None of the patients with grade 2 or 3 toxicity had a history of hepatic disease, eight patients concomitantly used potential hepatotoxic co-medication. Eight (8%) patients used leflunomide in combination with methotrexate, and one of these patients developed hepatotoxicity. No clinical signs of serious hepatotoxicity were recorded during follow-up. DISCUSSION: In 8.9% of the patients, grade 2 or 3 hepatotoxicity was recorded within the first year after the start of leflunomide therapy based on liver enzyme determinations. In the majority of the patients liver enzyme elevations occurred within the first 6 months of therapy and resolved during continued follow-up. None of the patients showed clinical signs of hepatotoxicity. CONCLUSION: Under continued monitoring of liver functions hepatotoxicity during leflunomide use does not seem to be a major problem in our population.
12199383 The use of a 95 degree blade plate and a posterior approach to achieve tibiotalocalcaneal 2002 Aug Various types of internal fixation have been used to achieve arthrodesis of both the ankle and subtalar joints. We have investigated the use of a standard 95 degree angled blade plate as a method of more rigid internal fixation to achieve arthrodesis of these joints. The purpose of this retrospective study was to review our clinical and radiographic results in adults using a blade plate applied through a posterior approach to fuse the ankle and subtalar joints. METHODS: Between April 1995 and June 2000, 10 tibiotalocalcaneal arthrodeses were performed using a posterior approach and a blade plate for internal fixation. There were 10 adults (five men and five women) whose average age was 64 years (range, 42 to 80 years). The indication for the procedure was severe pain which was unresponsive to nonoperative management in patients with arthritic joints. Preoperative diagnoses included six patients with post-traumatic arthritis, two with primary degenerative arthritis, one with rheumatoid arthritis, and one with post-polio deformity. An average of 1.7 previous operations had been performed on the affected ankle. RESULTS: Clinical and radiographic follow-up was performed for all patients at an average of 37 months (range, 12 to 71 months) postoperatively. All 10 patients achieved a solid fusion. The mean time to radiographic fusion was 14.5 weeks (range, 9 to 26 weeks). The operation resulted in plantigrade feet in all patients with an average tibia-floor angle of 2.3 degrees of dorsiflexion and an average of 5 degrees of hindfoot valgus. Patients had excellent pain relief, however function did not improve as much. Complications occurred in three patients. One patient required a small split-thickness skin graft for wound healing, one experienced a transient posterior tibial nerve neuropraxia, and one developed a deep venous thrombosis in the nonoperative leg at six weeks postoperatively. Three patients required removal of the blade plate because of discomfort, which promptly cleared. CONCLUSIONS: Arthrodesis provides excellent pain relief for patients with painful arthritic deformities of the ankle and subtalar joints. Using a posterior approach, a blade plate for internal fixation and bone grafts resulted in a solid fusion for all our patients. This method is particularly effective in large patients with a mild-moderate hindfoot deformity.
12610820 Remission in juvenile chronic arthritis: a cohort study of 683 consecutive cases with a me 2003 Mar OBJECTIVE: As continuity of care in our institution allows longterm followup studies, we reviewed the files of all consecutive patients with juvenile chronic (idiopathic) arthritis (JCA) followed since 1970 to establish the frequency of remission. METHODS: Charts of all patients with JCA were reviewed. Relevant variables were entered into a customized database. The presence of remission (lack of signs of disease activity in the absence of antirheumatic therapy for at least 6 mo) during the disease course and at the last visit was assessed. RESULTS: The cohort included 683 patients, 463 females and 220 males. According to the disease onset, 420 had oligoarticular, 108 polyarticular (23 rheumatoid factor positive), and 88 systemic disease; 67 had a juvenile spondyloarthropathy (SpA). For all 4 categories the mean followup period was about 10 years. At the last visit 224 cases were in remission (32.8%). Remission rate was scarcely influenced by age at disease onset, but differed in the different disease categories. Of the total group of 683 patients, 153 (22.4%) were lost to followup (no control for at least 2 years). For all 4 categories the remission rate at the last visit was higher in patients who had been lost to followup: 42.3% versus 29.0% for systemic onset JCA, 20.8% versus 16.5% for polyarticular onset JCA, 44.7% versus 33.6% for pauciarticular onset JCA, and 66.7% versus 26.8% for juvenile SpA. The probability of attaining remission decreased in proportion to delay in entering the tertiary care center (from 35.7% to 22.8%). The rate of remission reached its peak after 5-10 years of followup, after which the trend reversed. CONCLUSION: Childhood arthritis achieved remission in only about one-third of our cases, with differences among disease categories based on the diagnosis.
12847697 Human alpha-enolase from endothelial cells as a target antigen of anti-endothelial cell an 2003 Jul OBJECTIVE: To identify and recombine a protein of the human dermal microvascular endothelial cell (HDMEC) that specifically reacts with anti-endothelial cell antibody (AECA) in the serum of patients with Behçet's disease (BD), and to evaluate the usefulness of this protein in BD. METHODS: The proteomics technique, with 2-dimensional gel electrophoresis and matrix-assisted laser desorption ionization-time-of-flight (MALDI-TOF) mass spectrometry, was used to identify and recombine HDMEC antigen. Western blotting and enzyme-linked immunosorbent assay (ELISA) of recombinant protein isolated by gene cloning were performed on serum from healthy controls, patients with BD, and patients with other rheumatic diseases (rheumatoid arthritis, systemic lupus erythematosus, and Wegener's granulomatosis). RESULTS: Eighteen of 40 BD patients had serum IgM antibody to HDMEC antigen. The purified protein that reacted with AECA in BD patient sera was found to be alpha-enolase by 2-dimensional gel electrophoresis followed by immunoblotting and MALDI-TOF mass spectrometry. Recombinant alpha-enolase protein was isolated and refined by gene cloning. On Western blots, AECA-positive IgM from the sera of patients with active BD reacted strongly with recombinant human alpha-enolase. BD patient sera positive for anti-alpha-enolase did not react with human gamma-enolase. On dot-blotting, reactivity to human alpha-enolase was detected only in the IgM-positive group. Fifteen of the 18 AECA-positive sera that were positive for the HDMEC antigen showed reactivity to recombinant alpha-enolase IgM antibody by ELISA. CONCLUSION: The alpha-enolase protein is the target protein of serum AECA in BD patients. This is the first report of the presence of IgM antibodies to alpha-enolase in endothelial cells from the serum of BD patients. Although further studies relating this protein to the pathogenesis of BD will be necessary, alpha-enolase and its antibody may prove useful in the development of new diagnostic and treatment modalities in BD.
15636245 [Immunological phenomenons in the pathology of the tear film. Is cyclosporine the turn poi 2004 PURPOSE: The purpose of this study is to examine the new way in the casual treatment, resulting in the normalization of immunological processes on the ocular surface. MATERIAL AND METHODS: 5 patients (4 women and 1 men with mean age of 55.5 years, range 45 to 59 years) with Sjögren's syndrome--type dry eye, were included in the study. Patients received topically 0.05% cyclosporine, ophthalmic emulsion twice a day for a 6 months. The severity of patients complaints was evaluated according to the Dr. Mann Pharma/Bausch & Lomb's record. RESULTS: Schirmer test II values, and lyzamine green staining scores, were evaluated priori to treatment and 6 months after the start. At the beginning of the study (day 0) the Schirmer test II values ranged from 1 to 5 mm (mean--2.2 mm). Break-up-time ranged from 3 to 6 sec (mean--4.8 sec). In all of 5 cases lyzamine green staining scores were 9. On month 6th, all of 5 patients had significant differences, compared to their baseline measurements in term of above mentioned parameters. The Schirmer test II values ranged from 8 to 12 mm (mean--10.3 mm). Break-up-time ranged from 9 to 13 sec (mean--11.2 sec), lyzamine green staining scores ranged from 2 to 3 points (mean--2.7). CONCLUSIONS: 0.05% cyclosporine, ophthalmic emulsion had a beneficial effect both on the subjective and objective clinical parameters of dry eye patients. The final conclusions must be supported by more cases.
15581833 Genotypic analysis of the TGF beta-509 allele in patients with systemic lupus erythematosu 2004 Oct Transforming growth factor beta (TGFbeta) is a secreted protein present in the circulation and is a critical regulator of the body's immune system. TGFbeta is believed to control several components of the immune system and inhibit autoimmune reactions. Systemic lupus erythematosus (SLE) and Sjogren's syndrome (SS) are prototypical human autoimmune diseases characterized by the circulating autoantibodies directed against nuclear antigens and immune complex deposition in various tissues leading to target organ inflammation and damage. Although the etiology of SLE is unknown, it has been observed that patients with SLE have lower levels of circulating TGFbeta than healthy individuals. In addition, mice lacking the TGFbeta1 gene develop a severe autoimmune disease that has features of both SS and SLE. Polymorphisms in the TGFbeta1 gene may alter the mRNA expression levels and influence the plasma protein concentration. Of the known TGFbeta 1 polymorphisms, only the C-509T polymorphism in the promoter region has been shown to be significantly associated with the plasma concentrations of TGFbeta 1. In this study, we have conducted a blinded study to determine if the -509 TGFbeta1 gene polymorphism is associated with SS or SLE. Genomic PCR and RFLP analysis of a 441 bp sequence encompassing the -509 polymorphism of the TGFbeta gene indicated that there were no statistically significant clinical correlations.
15481820 Small fibers peroneal mononeuropathy in a patient with Sjögren's syndrome. 2004 Sjögren's syndrome (SS) is an autoimmune epithelitis characterized by lymphocytic infiltration of exocrine glands and epithelia in multiple sites. One third of the patients present with peripheral nervous system involvement. We describe the case of a woman aged 62 affected by a peroneal nerve mononeuropathy with painful disturbances secondary to a prevalent involvement of small fibers as demonstrated by electrophysiological investigations and skin biopsy. Asymmetric peripheral nerve involvement is not uncommon in SS, though, to our knowledge, it has never been reported of a mononeuropathy involving primarily small fibers.
14704553 Surgical treatment of dry eye syndrome: conjunctival graft of the minor salivary gland. 2004 Jan Despite the availability of efficient tear substitutes, many patients with dry eye syndrome experience severe corneal injuries and a subsequent loss of vision. Surgical techniques using mayor salivary glands to provide a substitute for tears have been reported; with this technique the drainage of saliva goes into the conjunctival fornix, permitting corneal and conjunctival humidification. The authors describe a new surgical approach in which minor salivary glands are autotransplanted into the conjunctival fornix by means of a graft of the intraoral mucosa-transporting salivary glands. This approach was used in a 56-year-old woman with a 2-year history of refractory and pharmacologically untreatable dry eye syndrome caused by Sjögren's syndrome. The right eye had more severe corneal and conjunctival lesions than did the contralateral one, so the treatment was planned in the right eye only. A weekly follow-up during the first 6 months confirmed the significant improvement of dry eye symptoms in the surgically treated eye. Three months after surgery, a biopsy was performed in the minor salivary gland graft, and the histologic findings revealed the presence of glandular acinus, duct with mucin content, and lymphocyte infiltration. The significant improvement obtained in this patient suggests that the secretion from the grafted salivary minor glands was better in promoting homeostasis of the ocular surface than are artificial tears. This may be explained by: (1) The lacrimal and salivary secretions contain biologically active constituents that may protect from infection and promote normal growth epithelium; (2) The secreted mucin is thought to coat the epithelial surface, reducing the high surface tension of the eye wetted by aqueous tears; (3) The thick secretions of the minor gland might act in reducing the evaporation of the underlying tear layer and form a hydrophobic barrier along the lid margin that can retain the lid margin tear string and prevent its flow onto the skin. Minor gland salivary autotransplant is a new surgical technique with effectiveness demonstrated in one patient, but the scientific explanation is not clear; additional experience with more cases could confirm the initial success.
11925884 [Adult-onset Still's disease accompanied by hemophagocytic syndrome at onset]. 2002 Feb A 43-year-old woman was admitted for examination of fever, an elevated transaminase level, LDH, skin eruption, sore throat and bicytopenia. As bone marrow examination revealed an increased proportion of histiocytes and active phagocytosis, hemophagocytic syndrome (HPS) was diagnosed. After admission, the peripheral blood counts recovered spontaneously and the HPS subsided, but other symptoms persisted and the neutrophil count increased. At this time, we diagnosed the patient as having adult Still's disease. All the symptoms disappeared after administration of prednisolone. The markedly increased concentrations of TNF-alpha and IFN-gamma in the peripheral blood at the time of HPS declined gradually, and the IL-6 concentration increased at the time of diagnosis of Still's disease. However, all of these concentrations normalized after administration of prednisolone. As HPS and Still's disease have a common etiology, and each shows high concentrations of IFN-gamma, IL 6 and TNF-alpha, the symptoms are similar in both diseases. In particular, a relationship between HPS and high concentrations of TNF-alpha and IFN-gamma is suspected.
15729880 [A case of subacute myelitis associated with primary Sjögren syndrome showing no MRI abno 2004 Dec We describe a case of Sjögren syndrome presenting with subacute transverse myelopathy that improved in response to steroid therapy. A 55-year-old woman with a history of NIDDM developed progressive sensory disturbance and weakness of the both legs, and micturition disturbance during a 6-month period, resulting in walking difficulty. On admission, neurological examination revealed paraplegia with extensor plantar reflex and sensory disturbance below Th 6 level. Lumbar puncture disclosed a normal cell count and a mild elevation of protein in the CSF. As subacute myelitis at the level of Th 6 was suspected, spinal plain and enhanced MRI was performed, which revealed no abnormalities. In contrast, tibial somatosensory evoked potential (SEP) could not be evoked while median SEP was almost normal, indicating thoracic cord involvement. She was diagnosed with primary Sjögren syndrome based on xerostomia, apple-tree appearance in parotid sialography, mononuclear cell infiltration in the salivary gland biopsied, and elevated anti-SS-A and SS-B antibody titers. We considered that her myelopathy was associated with Sjögren syndrome, and started steroid pulse therapy. After three courses of steroid pulse therapy followed by oral prednisolone, she showed clinical and SEP improvements. It is conceivable that the present case may represent Sjögren syndrome complicated with myelopathy that shows no MRI abnormality and well responds to steroid therapy. SEP can be a valuable tool for assessing the presence and severity of spinal cord involvement, and the efficacy of treatments.
15636257 [Microbiological evaluation of conjunctival sac in Sjögren syndrome patients]. 2004 PURPOSE: To evaluate bacterial flora of conjunctival sac in patients with Sjogren syndrome and compare it with the flora of healthy individuals. MATERIAL AND METHODS: Bacteriological test was performed on the swabs from the conjunctival sac of 15 patients with primary Sjögren syndrome (PS), 15 patients with secondary Sjögren syndrome (SS) and 30 healthy individuals, chosen at random. RESULTS: Most of the swabs, in every group, were aseptic. The bacteria found relatively often was Staphylococcus epidermidis or described as the Staphylococcus sp. coagulasonegative. Bacterias: Staphylococcus aureus, Corynebacterium or Enterobacteriaceae were found rarely. CONCLUSIONS: There is no significant difference between bacterial flora of conjunctival sac of patients with PS and SS, when compared to healthy individuals.
15328839 [Inclusion body myositis associated with chronic thyroiditis, Sjögren's syndrome and auto 2004 Jun A 67-year-old woman was admitted to our hospital, because of wasting of the thigh muscles. Muscular atrophy was confined to the thigh muscles, suggesting that she had quadriceps myopathy. Muscle biopsy from quadriceps muscle revealed characteristic findings, such as invasion of the endomysium and muscle fibers by inflammatory cells, the presence of rimmed vacuoles and ragged red fibers; thereby, she was diagnosed as having inclusion body myositis (IBM). Based on elevated titers of autoantibody as well as biopsy findings from salivary gland and liver, she was also diagnosed as having chronic thyroiditis, Sjögren's syndrome and autoimmune cholangitis. Currently, the pathogenic mechanism underlying IBM is not clarified, however, various factors have been suggested to contribute to it, such as viral infection or mitochondrial insufficiency. Although the pathogenic backgrounds underlying IBM are likely heterogeneous, an autoimmune-mediated mechanism may be related to the pathogenesis of IBM in the present patient.
12525207 Fungal infection of the epiglottis simulating a clinical malignancy. 2003 Jan Primary fungal infection of the larynx is rare. We present a case of primary laryngeal invasive fungal infection in a patient with Felty syndrome. The lesion in the epiglottis resembled a malignant process and the whole epiglottis was excised endoscopically. No neoplasia was found and the cultures of the epiglottis grew Candida glabrata. We propose that the diagnosis of laryngeal candidiasis be considered in adult patients with certain predisposing factors, eg, immunodeficiency syndromes, but the possibility of underlying malignancy must be ruled out.
15326117 An in vivo confocal masked study on corneal epithelium and subbasal nerves in patients wit 2004 Sep PURPOSE: The objective of the present study was to determine whether dry eye (DE) associated with primary Sjögren's syndrome (PSDE) and DE not associated with Sjögren's syndrome (NSDE) are related to an alteration of corneal innervation. METHODS: Eleven healthy volunteers younger than 60 years (normal [N] < 60 group), 10 healthy volunteers 60 years of age or older (N > or = 60 group), 11 patients with PSDE, and 10 patients with NSDE were studied. Epithelial and stromal density and subbasal and stromal nerves were investigated by confocal microscopy. RESULTS: The density of the superficial epithelial cells was 741 +/- 306 cells/mm2 in the PSDE group; 1022 +/- 331 cells/mm2 in the NSDE group; 1523 +/- 294 cells/mm2 in the N > or = 60 group, and 1529 +/- 341 cells/mm2 in the N < 60 group (P < 0.0001, ANOVA). The number of subbasal nerves was 2.8 +/- 1.2 in the PSDE group, 3.3 +/- 0.7 in the NSDE group, 3.1 +/- 0.9 in the N > or = 60 group, and 4.6 +/- 0.8 in the N < 60 group (P < 0.0001, ANOVA). The number of beadlike formations observed in the different groups was 387 +/- 62/mm in the PSDE group, 323 +/- 64/mm in the NSDE group, 182 +/- 63/mm in the N > or = 60 group, and 198 +/- 66/mm in the N < 60 group (P < 0.0001, ANOVA). A correlation was found between the number of subbasal nerves and age (P < 0.01) and between the number of subbasal nerves and Schirmer's test (P < 0.001, Spearman rho). CONCLUSIONS: Patients with DE show alteration in the corneal innervations. The demonstration of such alterations introduces new strategies for treatment of this frequent disease.
15138654 [Multiple autoimmune syndrome. Reynolds-syndrome (acral scleroderma, primary biliary cirrh 2004 May A female patient presented with acral scleroderma, Sjögren syndrome, antibodies specific for primary biliary cirrhosis and clinical as well as histological features of lichen planus and subacute lupus erythematosus. In addition an euthyroid Hashimoto thyroiditis was found. Her findings correspond to type II of the multiple autoimmune syndrome (MAS) and can be described as an association of Reynolds syndrome and the lupus erythematosus/lichen planus-overlap syndrome.
12741480 Transverse myelitis occurring in association with primary biliary cirrhosis and Sjogren's 2003 Apr Transverse myelitis (TM) as a manifestation of an autoimmune disorder is relatively rare. In Sjogren's syndrome (SS), the occurrence of TM is remarkably uncommon. Only three cases have been reported associated with primary biliary cirrhosis (PBC). Here we report the fourth case of TM occurring in association with SS and PBC. Patients with unexplained transverse myelitis require a careful search for an underlying etiology to include the findings of SS and PBC. The precise pathogenesis of TM in patients with SS is unknown. Most show good response to steroids. Cyclophosphamide and chlorambucil may be useful in those who respond poorly to steroids.
12195572 Fatal scleroderma renal crisis caused by gastrointestinal bleeding in a patient with scler 2002 May A 57-year-old female patient presented with a long history of overlapping autoimmune disease, including limited cutaneous systemic sclerosis, Sjögren's syndrome and primary biliary cirrhosis. Another unusual finding was that the mild skin involvement (limited cutaneous systemic sclerosis, subcutaneous calcinosis) was combined with serious internal organ involvement, including honeycombing and finally scleroderma renal crisis. The most important finding was, that two decades later she developed severe telangiectasia of the gastrointestinal and urinary tract. Furthermore, a specific type of vascular malformation, i.e. gastric watermelon stomach was also found. The chronic gastrointestinal bleeding primarily derived from a watermelon stomach caused protein overload, which provoked the onset of the scleroderma renal crisis that finally led to the patient's death.
12073793 [Still's disease in the adult]. 2002 Apr The Adult Onset Still's Disease (AOSD) is an entity of unknown origin characterised by fever, polyarthralgias or polyarthritis, a pharyngitis, a cutaneous eruption and elevation of leucocytes. We present observations of 17 patients diagnosed during the last thirteen years. Files of hospitalized patients with suspected AOSD have been examined in a retrospective study and Yamaguchi and Kahn's criterias applied in order to confirm the diagnosis. Treatments undertaken and the evolution have been noted. The sex ratio is 1.83; the middle age of intervening from 42 to 45 years. The middle delay for the diagnosis is 3 to 8 weeks. Fever, eruption and polyarthralgias are the most frequent clinical signs. Elevation of leucocytes, hepatic cytolysis and hyperferritinemia are usual. Constantly, inflammatory tests are increased and the rheumatoid factor and the antinuclear antibodies are negative. Yamaguchi's criterias have been met by all patients. The non steroïd anti-inflammatory drugs and corticoïds constituted the main treatment. In the evolutionary shield, 6 cases of remission have been noted. The other patients evolved toward the chronicity with either a systemic or an articular shape. In the two situations, a state of corticodependance got settled with necessity of association to immunosuppressive drugs. The diagnosis of AOSD is easily done on basis of Yamaguchi's criterias. Corticoïds can permit to control the bouts. There are 3 types of evolution: remission, chronic systemic or chronic articular shape.
14678568 The Functional Assessment of Chronic Illness Therapy (FACIT) Measurement System: propertie 2003 Dec 16 The Functional Assessment of Chronic Illness Therapy (FACIT) Measurement System is a collection of health-related quality of life (HRQOL) questionnaires targeted to the management of chronic illness. The measurement system, under development since 1987, began with the creation of a generic CORE questionnaire called the Functional Assessment of Cancer Therapy-General (FACT-G). The FACT-G (now in Version 4) is a 27-item compilation of general questions divided into four primary QOL domains: Physical Well-Being, Social/Family Well-Being, Emotional Well-Being, and Functional Well-Being. It is appropriate for use with patients with any form of cancer, and extensions of it have been used and validated in other chronic illness condition (e.g., HIV/AIDS; multiple sclerosis; Parkinson's disease; rheumatoid arthritis), and in the general population. The FACIT Measurement System now includes over 400 questions, some of which have been translated into more than 45 languages. Assessment of any one patient is tailored so that the most-relevant questions are asked and administration time for any one assessment is usually less than 15 minutes. This is accomplished both by the use of specific subscales for relevant domains of HRQOL, or computerized adaptive testing (CAT) of selected symptoms and functional areas. FACIT questionnaires can be administered by self-report (paper or computer) or interview (face-to-face or telephone). Available scoring, normative data and information on meaningful change now allow one to interpret results in the context of a growing literature base.
14642109 [Clinical features and misdiagnosis of anti-Jo-1 syndrome: analysis of 33 cases]. 2003 Sep 25 OBJECTIVE: To evaluate the clinical features and causes of misdiagnosis of anti-Jo-1 syndrome. METHODS: The data of 33 polymyositis (PM)/dermatomyositis (DM) patients with anti-Jo-1 antibody hospitalized from January 1997 to June 2002 in Peking Union Medical College Hospital were reviewed retrospectively. The initial symptom, clinical and laboratory characteristics, diagnosis, efficacy of therapy and follow-up were studied. RESULTS: 51.5% of the patients developed lung impairment and 24.2% of the patients developed arthritis/arthralgia before the onset of PM/DM, only 5 patients had the typical rash, and/or myalgia and muscle weakness as the initial symptoms (15.2%). Patients with anti-Jo-1 syndrome were usually misdiagnosed as with rheumatoid arthritis (27.3%), idiopathic pulmonary fibrosis (12.1%), and pneumonia (15.2%). Anti-Jo-1 syndrome was associated with inflammatory myositis (100%), interstitial lung disease (ILD) (93.9%), fever (60.6%), polyarthritis/polyarthralgia (54.5%), Raynaud's phenomenon (21.2%), and sclerodactyly (21.2%). The muscle symptom was relatively mild and the symptom of ILD was relatively severe. Treatment of corticosteroid plus immunosuppressive drugs was effective in achieving initial remission, while relapse was frequent. CONCLUSION: Few patients with anti-Jo-1 syndrome have the episode of classical myositis as initial symptom. Lung impairment, especially ILD, and joint involvement are more common in early phase of the disease.