ABSTRACT
Objective:To explore the clinical features and risk factors of in-hospital mortality in idiopathic inflammatory myopathies (IIM) patients.Results:We retrospectively analyzed clinical records of polymyositis (PM), classic dermatomyositis (CDM) and clinically amyopathic dermatomyositis (CADM) patients admitted to the First Affiliated Hospital of Zhejiang University from February 2011 to February 2019. The deceased group was defined as the patients who died in hospital or within 2 weeks after hospital discharge, while the survival group was defined as the survival patients. The clinical features were described. Risk factors for deceased patients were identified by logistic regression analysis.Results:The in-hospital mortality rate of IIM patients ( n=424) was 9.4%. The hospitalization time was longer in deceased group ( n=40) [0.9(0.5, 1.0) m vs 0.6(0.4, 1.0) m, Z=-2.159, P<0.05]. Ferritin [1170.8(757.6, 3 759.9) μg/L vs 374.9(182.1, 993.4) μg/L, Z=-4.665], red blood cell distribution width (RDW) [15.2(14.5, 16.3)% vs 14.4(13.5, 15.2)%, Z=-3.066], CRP con-centrations [11.3(4.4, 36.9) mg/L vs 5.1(1.8, 17.2) mg/L, Z=-2.667] and neutrophil-to-lymphocyte ratio (NLR) [10.1(5.5, 18.9) vs 4.2(2.6, 6.5), Z=-5.108] were higher in deceased group ( P<0.05). Proportion of patients with high levels of CEA (45.0% vs 12.5%, χ2=15.745), glutamyl transpeptidase (γ-GT) (55.0% vs 23.8%, χ2=11.578), fucosidase (AFU) (35.0% vs 10.0%, χ2=10.902) and with complications [including pro-gressive in-terstitial lung disease (ILD) (60.0% vs 16.3%, χ2=23.934), pulmonary infection (72.5% vs 20.0%, χ2=31.360), hemophagocytic lymphohistiocytosis (35.0% vs 1.3%, χ2=27.771) and low T3 syndrome (50.0% vs 17.5%, χ2=16.644) were higher in deceased group ( P<0.05). Steroid pulse therapy and intravenous immuno-globulin therapy were more common in deceased group. Higher on-admission disease activity [ OR=1.593, 95% CI(1.255, 2.022), P<0.001], progressive ILD [ OR=5.600, 95% CI(1.510, 20.772), P=0.010] and pulmonary infection [ OR=6.771, 95% CI(2.031, 22.574), P=0.002] were independent risk factors for death in IIM patients. In su-bsection analysis, pulmonary infection and respiratory failure were short-term adverse prognostic factors for IIM patients with progressive ILD, while heliotrope rash, progressive ILD and increased steroid dose therapy were short-term adverse prognostic factors for IIM patients with pulmonary infection. Conclusion:High disease activity at admission, progressive ILD and pulmonary infection are the independent risk factors for death in IIM patients. Therefore, it is necessary to closely monitor above indicators during hospitalization.
ABSTRACT
Objective To analyze the clinical characteristics of antiphospholipid syndrome (APS) in a cohort of Chinese patients. Methods From January 1996 to October 2009, 174 patients with defined APS were retrospectively studied,χ2 test was selected. Results The cohort consisted of 151(86.8%) female and 23 (13.2%) male. Mean age at study was (42±13) years (range: 14-75 years). Thirty-one (17.8%) patients had primary APS, 143 (82.2% ) patients had APS associated with other diseases, including 138 cases of systemic lupus erythematosus (SLE). One hundred and forty-one (81.0%) had thrombosis episodes, among which the most common presenting manifestations were stroke (27.6%), deep venous thrombosis (27.6%) and pulmonary embolism (6.3% ). Stroke was more prevalent in Chinese than European patients. Forty-five patients (31.9%) had thrombotic recurrences and 62 patients (44.0%) had more than two sites of thrombosis. Patients with primary APS had higher rates of rethrombosis than those with secondary APS (P<0.05). Fetal morbidity rate of 126 married women in our study was 50.0%. Seven of APS patients with APS nephropathy were characterized by thrombotic microangiopathy. The presence of anticardiolipin antibodies was detected in 112 patients (64.4% ), anti-beta-2 glycoprotein Ⅰ antibodies in 103 patients (59.1%) and lupus anticoagulant in 50 patients (28.7%). Conclusion APS is characterized by recurrent thrombosis, pregnancy morbidity and positive antiphospholipid antibodies tests. Stroke, deep venous thrombosis and pulmonary embolism are the most common manifestations both in Chinese and European patients. Rethrombosis is more prominent in primary APS. Thrombotic microangiopathy is one of the most common histological changes of APS nephropathy. Multiple tests for aPL are proposed.