RESUMO
Objective:To study the clinical and immunological features of two case of rare antisynthetase syndrome (ASS), so as to improve the level of diagnosis and treatment.Methods:Two cases with rare antisynthetase syndrome admitted to the First Affiliated Hospital of Zhengzhou University from July 2020 to August 2022 were collected.Results:The two rare ASS were anti-Zo antibody and anti-Ha antibody positive patients, both of which had interstitial lung disease (ILD) as the main clinical manifestation and positive anti-Ro52 antibody. Two rare antisynthetase autoantibodies manifested cytoplasmic ANA indirect immunofluorescence (IIF) staining pattern, but it is different from the cytoplasmic dense speckled pattern of several common ASS antibodies. After treatment with glucocorticoids and immunosuppressants, case 1 died of respiratory failure due to a long course of disease and late diagnosis, the lung lesions of case 2 improved significantly.Conclusion:When encountering the cytoplasmic ANA fluorescent pattern in ILD patients, especially with anti-Ro52 antibody, it is necessary to screen more myositis specific antibodies to rule out the possibility of rare ASS.
RESUMO
Objective:To study clinical features of rheumatoid arthritis (RA) patients with peripheral neuropathy (PN).Methods:The clinical data of 46 RA patients with PN in the First Affiliated Hospital of Zhengzhou University from August 2012 to August 2019 were retrospectively analyzed, including clinical manifestations, laboratory and imaging results, previous treatment, treatment and clinical outcome. The other 92 RA patients without PN at the same period were selected as controls.Results:In RA patients with PN, the male to female ratio was 1∶2.1 with an average age (59.1±11.8) years. The course of RA and PN was 102.0 (19.0-156.0) months and 4.2 (0.7-5.5) months respectively. Numbness (84.8%, 39/46) and muscle weakness (21.7%, 10/46) were the most common symptoms. According to results of electromyography, polyneuropathy (60.0%, 27/46) was the predominant manifestation, followed by mononeuritis multiplex (31.1%, 14/46). Compared to RA patients, rheumatoid factor (RF) ( P<0.001) and the percentage of cutaneous vasculitis ( P=0.042) were higher in RA patients with PN. Logistic regression analysis revealed significant correlation between RF>178.4 IU/ml ( OR＝5.626, 95% CI 2.509-12.618, P<0.001) and development of PN. Paresthesia in 27 patients (58.7%, 27/46) were relieved after treatment of high dose glucocorticoid and immunoglobulins (IVIG). Twelve patients were followed up regularly and the mean duration of follow-up was 17.0(4.8-52.8)months. Paresthesia in 10 (10/12) patients were relieved compared to that at discharge, 1 (1/12) patient achieved complete remission. Conclusion:Numbness and muscle weakness are the common symptoms in RA patients with PN and polyneuropathy is the main type. RF>178.4 IU/ml is correlated with the development of PN in RA patients. Intensive treatment such as high dose glucocorticoid and IVIG are effective.