Acute Polymyositis/systemic Lupus Erythematosus Overlap Syndrome with Severe Subcutaneous Edema and Interstitial Lung Disease

2014 ◽  
Vol 21 (1) ◽  
pp. 25
Author(s):  
U-ram Jin ◽  
Kyu-Sung Kwack ◽  
Kyung-Joo Park ◽  
Ji-Eun Kwon ◽  
Si-Yeon Kim ◽  
...  
2019 ◽  
Vol 8 (2) ◽  
pp. 40-43
Author(s):  
Gina Amanda ◽  
Prima Belia Fathana ◽  
Dianiati Kusumo Sutoyo

Author(s):  
lou deneuville ◽  
Karim Sacre ◽  
Marie-Pierre Debray ◽  
Pascale Nicaise-Roland ◽  
Bruno Crestani ◽  
...  

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 628.2-629
Author(s):  
I. González Fernández ◽  
C. Moriano ◽  
E. Diez Álvarez ◽  
X. E. Larco Rojas ◽  
A. López Robles ◽  
...  

Background:Lung involvement1 in Systemic Lupus Erythematosus (SLE) is heterogeneous, with the pleura being more frequently affected. Associated diffuse interstitial lung disease (ILD) is rare (3-13%) with difficult diagnosis and therapeutic management.Objectives:Assess the main clinical-epidemiological characteristics of patients diagnosed with SLE with ILD in a third level hospital and analyze the possible relationship between them.Methods:Descriptive observational study of patients diagnosed with SLE (SLICC criteria) with ILD in our hospital between 1973 and 2020. The following clinical-epidemiological characteristics were evaluated such as sex, mean age at diagnosis, presence of cardiovascular risk factors (CVRF), smoking, baseline cardiac and respiratory comorbidities, laboratory markers (autoimmunity, CRP and vitamin D), baseline SLEDAI, manifestations of SLE, interstitial pattern on HRCT, correlation with conventional radiology (Rx), respiratory function tests, clinical and physical examination at diagnosis.Results:455 patients diagnosed with SLE were included, of whom 20 had ILD (4.4%). 65% were women with a mean age at diagnosis of 63 ± 16.23 years. 30% presented ILD as the first clinical data for the diagnosis of SLE. 30% had jobs with exposure to chemicals. 25% were ex-smokers and 10% were active smokers. 10% had associated CVRF, highlighting HT (35%). 30% had valve disease on echocardiography such as mitral regurgitation (30%) and tricuspid regurgitation (20%). Only 10% had associated bronchopathy. 35% and 25% associated, respectively, pleural serositis and secondary Sjogren’s syndrome. Arthritis and skin involvement were documented in 60% for both clinical domains. 40% had SLEDAI> 6.At the diagnosis of ILD, 90% had symptoms such as dyspnea (75%) and cough (60%). 80% had crackles on examination. 85% had alterations in the X-ray and 100% in the CT scan with the NINE patterns as the main one (65%) with progression at 2 years despite treatment in 25%. Spirometry differentiated an obstructive pattern (25%), restrictive (20%) and normal (55%) with a tendency to stability at one year (40%) with the prescribed treatment. 90% had a decrease in diffusion, the majority being mild (67%). Analytically, 67% had elevated levels of CRP and hypovitaminosis D; 40% elevated DNA titers, 50% hypocomplementemia and 60% ENAS positive, highlighting Ro (45%); La (15%) and RNP (15%). Leukopenia and thrombopenia (30% and 10% respectively) were also observed.In our study, we did not find any statistical significance between the variables analyzed, due to the small sample size.Conclusion:1)ILD is an infrequent manifestation in SLE, it usually associates respiratory symptoms and pulmonary functional repercussions with the diagnosis. The most characteristic radiological pattern is the NINE.2)In our study, ILD was more frequently associated with women and joint or skin symptoms, as well as with data on immunological activity, without statistical significance.References:[1]Aguilera-Pickens G, Abud-Mendoza C. Pulmonary Manifestations in Systemic Lupus Erythematosus: Pleural Involvement, Acute Pneumonitis, Chronic Interstitial Lung Disease and Diffuse Alveolar Hemorrhage. Clinical rheumatology. Vol.14 Issue. 5. Pages 294-300 (September-October 2018).Disclosure of Interests:None declared


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