Interstitial Lung Disease Complicated By Connective Tissue Disease

BACKGROUND:The incidence of interstitial lung disease increased year by year. Connective Tissue Disease infringes the alveolar epithelium, alveolar basement membrane, interstitial cavity, and terminal bronchioles, cause interstitial lung disease. The reason of interstitial lung disease caoused by connective tissue disease takes a large proportion of all the reasons.As the Spreading and increasing of pathology, laboratory testing instruments, the diagnosis rates of these diseases increased gradually. We describe the clinical diagnosis of these diseases, laboratory tests, X-ray findings, then summarize the clinical manifestations, laboratory examinations and treatment characteristics, are as follows.Objective:This article has reviewed of the literature and researched advances of ILD complicated by CTD, analyzed to summarize the clinical manifestations, laboratory examinations and treatment characteristics of 50 patients with ILD complicated by CTD. Aims to improve the experience of the diagnosis and treatments of ILD complicated by CTDResults:The major respiratory symptoms of CTD associated ILD are cough, dyspnea, also can be accompanied by fever, cough and chest pain. All the patients have cough and dyspnea. In which there are 17 cases with fever (34%),34 cases with expectoration (48%),2 patients with chest pain (4%), but none of hemoptysis. In which patients with fever, there are 2 cases of RA, 5 cases of ANCA associated vasculitis,3 cases of PM/DM,4 cases of SLE,3 cases of MCTD. There are 23 cases with Crackles (46%). (2) Laboratory examinations:All the 50 cases of CTD have abnormal erythrocyte sedimentation rate,38 cases have abnormal C-reactive protein (76%),27 cases have abnormal rheumatoid factor (54%). Different types of CTD have its own characteristics:Rheumatoid factor in patients of RA was higher, between 1000 to 4000IU/L, CRP and ESR correlated with the severity of the disease (Clinical symptoms, arterial blood gas analysis, CT performance). Anti-SSA antibody, anti-SSB antibody and ANA were strongly positive in all the cases of pSS. The ANA of 4 cases of SSc both were positive, of which 2 with Scl-70 antibody positive, The pulmonary lesions in the patients with Scl-70 antibody positive is more serious,compared with the patient with Scl-70 antibody negative, Scl-70 antibody is a marker of systemic scleroderma antibodies; Cytosolic anti-neutrophil cytoplasmic antibody (c-ANCA) are positive in patients of ANCA associated vasculitis, complement reduced,but globulin increased, rheumatoid factor is positive in some patients of them. It is reported that c-ANCA may be indicators to reflect disease activity. CK, LDH, AST, ALT increased in all the cases of PM/DM, among them there are 4 cases with Jo-1 antibody positive. Renal damage is obvious in all the cases of SLE, the Specificity of Anti-double-stranded DNA antibod antibody (dsDNA), anti-Smith antibody is more than 90%. (3) Chest CT performance:the performances of CT of 50 patients include interlobular septal thickening, ground glass shadow, honeycomb-like shadow, pleural off the assembly line, pleural thickening. Ground glass is the most common, followed by septal thickening, grid-like and honeycomb-like shadow. The performance of lung legion show a mixed performance. Different types of CTD Associated ILD in imaging have their own characteristics. (4) Pathology:21 patients underwent a bronchoscopy and pathology. There are 15 pathology results revealed chronic inflammation, interstitial lymphocytic infiltration, the CT of these patients as mostly ground glass and a small amount of grid shadow, honeycombing.That is considered consistent with NSIP. The other 6 Pathology results revealed acute and chronic inflammation, interstitial neutrophil infiltration and lymphocytic infiltration, CT with a lot of honeycomb-like shadow, that may be the UIP. (5) Blood gas analysis:5 cases is hypoxemia,45 cases is type I respiratory failure, there are 12 patients have died. Oxygen pressure is less than 30mmHg. (6) 50 patients were therapyed by corticosteroid in different methods. There are 12 patients died during hospitalization, including 4 cases of ANCA associated vasculitis associated ILD,3 cases of SLE-related ILD,4 cases of PM/DM related ILD, 1cases of SSc-related ILD.13 cases of PSS patients received 2-6 months of corticosteroid therapy,9 cases ground glass is almost all absorbed, 1cases with no significant changes,3 cases of pulmonary bullae appeared.We followed up 3 patients of RA, their lung lesions deteriorates gradually.Conclusions:(1) ILD complicated by CTD have a higher prevalence, the main respiratory symptoms are cough, Dyspneadyspnea, also accompanied by fever, cough and chest pain. CTD easily violates cardiovascular, kidney, liver, and other system.(2) The erythrocyte sedimentation rate, rheumatoid factor, C-reactive protein will be significantly increased in CTD patients with lung lesions appeared. In the acute phase, Different types of CTD have their own specific indicators to response their condition changes,patients of ILD should accept rheumatic series of checks to know a clear cause.(3) Lung CT manifestations and pathological findings have the important guiding significance for the treatment and prognosis of CTD related ILD. UIP has poor response to the hormone but, NSIP has sensitive response to the hormone therapy. (4) Glucocorticoids and immunosuppressants should be used as soon as possible to antagonize fibrosis.