BackgroundAutoantibodies (abs) directed against Ro52/TRIM21 are common in systemic sclerosis (SSc) but their clinical significance remains uncertain. Some reports suggested that anti-Ro52/TRIM21 abs-positive SSc patients present interstitial lung disorders (SSc-ILD). However, it is not clear whether positive for anti-Ro52/TRIM21 related to other clinical manifestations in patients with SSc.ObjectivesThe aim of this study is to clarify the prevalence of anti-Ro52/TRIM21 abs in patients with SSc. Then, we investigated the clinical manifestations between anti-Ro52/TRIM2 abs-positive and negative patients with SSc.MethodsThis study is a retrospective case control study. The medical records of 42 patients who were diagnosed as having SSc admitted to our hospital were reviewed. We evaluated the clinical manifestations at the first-onset of SSc such as Rodnan skin score, digital ulcer, abnormal subcutaneous calcification, esophageal reflux, pulmonary hypertension, myositis and arthralgia. Co-existing rheumatic diseases were also reviewed such as Sjogren syndrome (SjS), rheumatoid arthritis (RA), and polymyositis. All subjects underwent SSc –associated abs testing using EUROLINE immunoblot assay. The autoantibodies include anti-Scl-70, anti-centromere A and B (CENP-A, CENP-B), anti-RNA polymerase III (RP-11, RP-155), anti-fibrillarin (U3RNP), anti- 90-kd nucleolar protein (NOR-90), anti-Th/To, anti-PM/Scl-100, anti-PM/Scl-75, anti-Ku, anti-platelet-derived growth factor receptor (PDGFR), and tripartite motif–containing protein 21(Ro-52). The association between clinical features and autoantibody profile was evaluated. Diagnosis of ILD was evaluated by chest high-resolution CT (HRCT).ResultsThirty-five patients with SSc are female (87%). Twenty-one and nineteen patients with SSc are positive and negative for anti-Ro52/TRIM21 abs, respectively (47 v.s 52%). There is no difference in population of diffuse type of SSc between anti-Ro52/TRIM21 abs-positive and negative SSc patients. In addition, the prevalence of ILD is not different between two groups (57 v.s 52%, p=0.76). The prevalence of SjS is tended to be higher in anti-Ro52/TRIM21 abs-positive SSc patients than in negative SSc patients (68 v.s 33%, p=0.05). Unexpectedly, 57% of anti-Ro52/TRIM21 abs-negative SSc patients present poly-arthralgia at the onset of SSc. In addition, there were not any complications such as osteoarthritis and RA in anti-Ro52/TRIM21 abs-negative SSc patients. The prevalence of arthralgia was higher in anti-Ro52/TRIM21 abs-negative SSc patients than in positive SSc patients (p=0.02).ConclusionsThe prevalence of anti-Ro52/TRIM21 abs in SSc patients of this study seems to be high compared to other reports. In addition, there seems to be no difference in the prevalence of ILD regardless of existence of anti-Ro52/TRIM21 abs in SSc patients. Anti-Ro52/TRIM21 abs-negative SSc patients were tended to present poly-arthralgia. This musculoskeletal disorder of anti-Ro52/TRIM21 abs-negative SSc patients may be not associated with other rheumatic diseases s...
Rationale:Kawasaki disease (KD) is an acute febrile illness predominantly affecting children less than 5 years of age and characterized by systemic inflammation in all medium-sized arteries. Adult-onset KD (AKD) is rare with only 105 case reports published. Recently, the efficacy of infliximab (IFX) for patients with refractory KD has been demonstrated.Patient concerns:A previously healthy 24-year-old man was admitted because of a persistent fever, and elevated serum level of AST, ALT, LDH, and CRP.Diagnosis:The patients met the diagnostic criteria for KD based on the findings of persistent fever, polymorphous exanthema, unilateral cervical lymphadenopathy, non-purulent palpebral conjunctivitis and membranous desquamation. Echocardiogram revealed the dilatation at the proximal sites of the right coronary artery (7.9 mm) and left anterior descending artery (5 mm). The patient was treated with high-dose intravenous immunoglobulin (1 g/kg/day for 2 days) and ASA (100 mg daily). However, his fever and arthralgia persisted.Interventions:He was administered single 5 mg/kg doses of IFX.Outcomes:He became afebrile the next day and his arthralgia improved.Lessons:We report the first case of administration of IFX in a patient with AKD refractory to intravenous immunoglobulin (IVIG), and successful reduction of systemic inflammation. However, the effectiveness of IFX in the regression of coronary artery aneurysm remains to be determined.
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