Association of acute anterior uveitis with disease activity, functional ability and physical mobility in patients with ankylosing spondylitis: a cross-sectional study of Chinese patients in Taiwan.

Acute anterior uveitis (AAU) is the most frequently extra-articular manifestation of ankylosing spondylitis (AS). To investigate whether AAU has an association with disease activity, functional ability and physical mobility in AS patients, 146 Chinese AS patients in Taiwan were enrolled in a cross-sectional study. These patients fulfilled the 1984 modified New York criteria and visited the Outpatient Department of the Veterans General Hospital-Taipei from April 2004 to July 2005. Patients completed questionnaires assessing disease activity [Bath Ankylosing Spondylitis Disease Activity Index (BASDAI)], functional ability [Bath Ankylosing Spondylitis Functional Index (BASFI)] and patient's global assessment [Bath Ankylosing Spondylitis Patient Global Score (BAS-G)]. Meanwhile, physical examinations were performed, including Schober test, finger-to-floor, lateral spinal flexion, occiput-to-wall and chest expansion. The history of AAU was accepted only if diagnosed by an ophthalmologist. The prevalence of AAU in this Chinese AS cohort was 15.8% (23/146). Patients with AAU had a significantly higher BASDAI than those without [absolute differences=0.96, 95% confidence intervals (CI): 0.35-1.88]. Additionally, patients with AAU had significantly increased BASFI than those without (absolute differences=1.46, 95% CI: 0.33-2.59). Moreover, there was advanced limitation of physical motility in patients with AAU, including finger-to-floor, occiput-to-wall distances and Schober test, (95% CI: 3.89-16.95 and p=0.046, respectively). Disease duration mildly correlated with BASFI (r=0.24, p=0.003) but not with BASDAI (p=0.838). There was no difference of disease duration between patients with and without AAU (p=0.343). These results suggested that the presence of AAU in AS patients may be associated with higher disease activity, poor functional ability and advanced physical impairment.

Undifferentiated spondyloarthropathy in Chinese patients.

BACKGROUND: Undifferentiated spondyloarthropathy (USpA) is a unique group in spondyloarthropathy (SpA). This study will investigate the clinical and laboratory characteristics of USpA in the Chinese population. METHODS: Forty two patients with USpA were enrolled from our rheumatology outpatient facility in this retrospective study. SpA was diagnosed according to the European Spondyloarthropathy Study Group (ESSG) criteria. Patients were considered having USpA when they had SpA but did not meet the criteria for the diagnosis of ankylosing spondylitis (AS), Reiter's syndrome or reactive arthritis (ReA), psoriatic arthritis (PsA), and inflammatory bowel diseases (IBD)-related arthritis, etc. Laboratory tests included erythrocyte sedimentation rate (ESR), serum C-reactive protein (CRP), immunoglobulin A (IgA), and human leukocyte antigen B27 (HLA-B27). RESULTS: Among the 42 USpA patients, the ratio of men to women was 1.47:1, and the age at onset was 32.33 +/- 10.83 years old. Approximately 61.9% of patients had peripheral arthritis, 30.95% had uveitis, and 64.29% had positive HLA-B27. Among these female USpA patients, compared to males, there is a trend of older age at disease onset, higher percentage of HLA-B27 positive, more peripheral arthritis and uveitis, longer disease duration, and higher level of ESR, and IgA and CRP in serum. The items reaching significant difference between males and females were longer disease duration (p < 0.001), higher level of ESR (p < 0.001), and serum IgA (p = 0.03). There was no significant difference in clinical and laboratory characteristics between HLA-B27-positive and -negative groups. CONCLUSIONS: Studies on USpA patients have not been reported in the Chinese population. In this study, we demonstrate the unique demographic characteristics, clinical and laboratory data of USpA in the Chinese population. These findings should be confirmed by analyzing larger number of patients and longer time for further follow-up. Such studies are crucial to understand the pathogenesis of USpA and evaluate its prognosis.

Pulmonary arterial hypertension in autoimmune diseases: an analysis of 19 cases from a medical center in northern Taiwan.

BACKGROUND AND PURPOSE: Pulmonary arterial hypertension (PAH), a serious complication of autoimmune diseases, has rarely been reported in Taiwan. METHODS: Nineteen patients with various autoimmune diseases diagnosed with PAH at Taipei Veterans General Hospital from 2002 to 2004 were enrolled; the underlying autoimmune diseases included systemic lupus erythematosus (n = 6), primary Sjögren's syndrome (n = 5), systemic sclerosis (n = 4), adult-onset Still's disease (n = 2), and mixed connective tissue disease (n = 2). The characteristic manifestations of underlying autoimmune diseases and the clinical features of PAH were analyzed. RESULTS: There were 16 female and 3 male patients. The median age at onset of PAH was 44 years and the mean right ventricular systolic pressure (RVSP) was 67.9 mm Hg. Patients without pneumonitis had a significantly higher RVSP value than those with pneumonitis (77.5 +/- 24.3 vs 54.8 +/- 18.4 mm Hg, p=0.041). Four out of 7 patients (57.1%) with RVSP >or=80 mm Hg and 1 out of 12 patients (8.3%) with RVSP <80 mm Hg died. In all of the 19 patients, the severity of RVSP was significantly correlated with serum uric acid (UA) level (r = 0.686, p=0.001). Among the PAH patients without pneumonitis, the severity of RVSP inversely correlated with the diffusion capacity of the lung for carbon monoxide (DLCO) [r = -0.856, p=0.003]. The characteristic manifestations of underlying autoimmune diseases included a high incidence of Raynaud's phenomenon (15/19, 78.9%), a high titer of antinuclear antibody (13/17, 76.5%), positive anti-ribonucleoprotein antibody (8/15, 53.3%), hypergammaglobulinemia (15/19, 78.9%), hyperuricemia (13/19, 68.4%), and less renal involvement. CONCLUSIONS: PAH in autoimmune diseases could be potentially fatal with characteristic manifestations. Moreover, RVSP correlated directly with serum UA level and inversely with DLCO.

Risk of retinal vein occlusion in patients with systemic lupus erythematosus: a population-based cohort study.

BACKGROUND/AIMS: To investigate the risk of developing retinal vein occlusion (RVO) in patients with systemic lupus erythematosus (SLE). METHODS: In this retrospective population-based cohort study, 6756 patients with SLE and 40 536 controls without SLE (1:6) were selected from the Taiwan National Health Insurance Research Database claims from 2001 to 2006. RESULTS: The incidence of RVO was 3.46-times higher in the SLE group than in controls (5.61 vs 1.62 per 10 000 person-years) (adjusted HR=3.883, 95% CI 2.299 to 6.558) calculated using Cox proportional hazard regression. Age was an independent risk factor for RVO, with adjusted HRs of 4.842 for individuals aged ≥50 years compared with those 0-49 years. For each age subgroup, the risk of RVO was significantly higher only in the 0-49-year-old SLE group than in controls. In the 0-49-year-old SLE group the incidence of developing RVO was 7.92 times higher (95% CI 3.60 to 17.45) in the SLE group than in the 0-49-year-old controls. In women with SLE, the incidence of developing RVO was 3.33-times higher (95% CI 1.88 to 5.90) than in female controls. CONCLUSIONS: SLE was significantly associated with an increased risk of developing RVO.

Multicentric reticulohistiocytosis presenting with destructive polyarthritis, laryngopharyngeal dysfunction, and a huge reticulohistiocytoma.

Multicentric reticulohistiocytosis (MRH) is a rare multisystemic disease presenting with skin lesions and erosive polyarthritis and is often associated with malignancy. We describe a 60-year-old woman with diffuse papulonodular skin eruptions and progressive osteolytic bone damage over the bilateral hands, humeral head, and acromioclavicular joints within 2 years. Moreover, dysphagia and a hoarse voice occurred in this patient and an unusual huge mass-reticulohistiocytoma--developed over the left upper back. Tissue biopsy of the skin lesions, laryngeal nodules, and this large mass showed infiltration of numerous CD68(+) histiocytes and multinucleated giant cells with abundant eosinophilic ground-glass cytoplasm. Combination therapy with steroids and methotrexate improved her cutaneous, joint, and laryngopharyngeal symptoms. The large reticulohistiocytoma resolved with methylprednisolone pulse therapy. This polyarthritis, which can be confused with rheumatoid arthritis, can be diagnosed by careful immunohistochemical examination of biopsies. To prevent the irreversible disease process, early and aggressive therapy is necessary.

Middle lobe syndrome as the pulmonary manifestation of primary Sjögren's syndrome.

Middle lobe syndrome - recurrent atelectasis and/or bronchiectasis involving the right middle lobe and/or lingula - has, up to now, not been reported as the pulmonary manifestation of primary Sjögren's syndrome. We describe a patient in whom lymphocytic bronchiolitis in the atelectatic lobes was proved histologically from two separate transbronchial biopsies. The atelectasis responded well to glucocorticoid treatment, suggesting that the peribronchiolar lymphocytic infiltrates may have played an important role in the development of middle lobe syndrome in this patient.