Clinical Analysis Of118Cases Of Connective Tissue Disease-associated Interstitial Lung Disease

Background:Connective tissue disease is a kind of systemic disease which is charactered by theimmune mediated organ damage. It often involves many systems and organs. Atpresent, because e of the use of high-resolution computed tomography, the diagnosticlevel of connective tissue disease-associated interstitial lung disease (CTD-ILD)improves a lot, but the morbidity and mortality is at all-time high. so CTD-ILD hasbecome a research hotspot in recent years.Objective:Accumulate the clinical data about CTD-ILD, discuss the clinical characteristicsof CTD-ILD and improve the level of understanding, diagnosis and treatment ofCTD-ILD.Methods:We retrospectively analyze the clinical manifestations, common blood counts,acute phase reactants, immune biological indicators, results of transbronchial lungbiopsy, possible pathological classifications, treatments and prognoses of118patientswith CTD-ILD from September2009to October2013received by the respiratorydepartment of the second hospital attached to Jilin University, and some of thepatients are followed-up.Results:1.Clinical manifestations：23cases (19.5%) have dry cough,90cases(76.3%) haveprogressive dyspnea,49cases (41.5%) have fever,23cases (19.5%) expect purulentsputum,5cases (4.23%) occur hemoptysis,29cases (25.6%) catch arthralgia,11cases (9.3%) catch myalgia. As to the clinical signs:97cases (82.2%) have velco rale,7cases (6%) have dry rale,6cases (5.1%) get skin sclerosis and8cases (6.8%) getskin rash of erythema.2.Onset modality:11cases onset with pulmonary manifestations as initial symptoms; CTD and ILD are diagnosed at the same time in46cases; As to the rest61cases, there is a long interval from the beginning of CTD to the definite diagnosisof ILD.3.Laboratoty tests:（1）common blood counts: the number of cases which own anelevated total number of WBCs or an increased ratio of the neutrophils is58, and47cases own the normal total number and classification of WBCs, while the total WBCsdecrease in13cases. There is no significant difference for the elevated white bloodcell count in the patients who have been treated with glucocorticoid or not, neither arethe cases who have been treated with glucocorticoid and immunosuppressor.27casesdevelop anemia,70%of whom is a mild anemia. And the ratio of patients withanemia is higher in RA-ILD, SLE-ILD and MPA-ILD.(2)Acute phase reactants(blood sedimentation, C-reactive protein)：62cases (52.5%) have an increase in CRP,of which16cases(13.6%) have an increase of WBC number and49cases(41.5%)have an increase of ESR at the same time.(3) Biochemical indicators: transaminaseelevates in35cases, serum creatinine elevates in10cases,creatine kinase（CK)elevates in5cases, CK and CK-MB elevate at the same time in3cases.(4) D-dimer:41cases have the results of D-dimer, in which there are36cases have an increase ofD-dimer, ranging between277to6043ug/L, and the average value is1874ug/L. Thereare26CTD-ILD cases from Feb2013to Oct2013totally, D-dimer was tested in24cases, and22cases own an elevated D-dimer.The average value is1670ug/L, but wedo not find the patients who encoutered thromboembolic events.(5) Rheumatismrelated checks: there are28cases have the results of RF, and24（85.7%）of that arepositive; there are24cases have the results of anti-CCP body, and19（79.2%）of thatare positive. In the study, the anti-Scl-70antibody of8patients with SSc-ILD ispositive in5cases;5patients are positive for anti-U1RNP antibody. The anti-SSAantibody of patients with SS-ILD is positive in12cases, of whom8cases (75%)havea more severe lesion in chest CT such as grids, honeycombs and extensiveground-glass opacity. The anti-JO-1antibody of patients with PM/DM-ILD is positivein4cases, in which one case is characterized by rapid-progress acute interstitialpneumonia. Both anti-ds-DNA antibody and anti-Sm antibody are negative in7 patients with SLE-ILD. In13cases with MPA-ILD, p-ANCA is positive in8casesand all p-ANCA recognises MPO; c-ANCA is positive in3cases and recognisesMPO in2cases and PR3in1case; p-ANCA, c-ANCA,MPO,PR3are all positive in1case; ANCA is negtive in3cases.4. Possible pathological classification: according to the clinic-imaging-pathological classification (lack of lung biopsy), we classify the118patients withCTD-ILD into possible pathological types according to our experience as far asposssible. There are38cases conforming to the changes in UIP and41casesconforming to the changes in NSIP, COP in3cases, AIP in2cases, LIP in2cases,but there are no cases conforming to DIP or RB-ILD.30cases can not be classified.UIP-like changes is more than NSIP in patients with RA-ILD,MPA-ILD and OS-ILDand is the mose obvious in RA-ILD. NSIP-like changes is primary in the other typesof CTD-ILD.5. Treatment and follow-up: Taking account of the factors such as age, basicdisease, nutritional status and the possible pathological classifications ect, weadministrate glucocorticoid and (or) immunosuppressant to the patients. During thetreatment course,31patients (26.3%) incurred diverse adverse drug reactions, inwhich9patients were clinically diagnosed with pulmonary fungal disease because ofthe much more likely restrain of immunologic function. All of the patients,7died inthe hospital,17died out of the hospital,25cases lose the follow-up, so the mortalityis25.8%.Conclusion:1.CID-ILD has a very high incidence, the diagnostic level has improved year byyear because of the continous improvement of check methods, but the disability rateand mortality is at an all-time high.2. It can be found clinically that part of cases who were diagnosed as IIP will bedefinitely diagnosed as CTD-ILD in several or decades of years,so the CTD casesonset with pulmonary manifestations as initial symptoms are not rare.3.ILD onsets invisibly in cases with a CTD history for decades of years. Because of the atypical or abscent symptoms patients will not see doctors timely, so whenCTD-ILD are diagnosed at late stage their prognosis is always poor.4.We classify the118patients with CTD-ILDA according to the priorclinic-imaging-pathological classification experience, and find different CTD-ILDhave its respective tendency to the pathological classifications. UIP-like changes isprimary in patients with RA-ILD,MPA-ILD and OS-ILD,NSIP-like changes isprimary in the other types of CTD-ILD. LIP cases are most common in SS-ILD.CADM cases always tend to acute progressive and refractory LID.5.The acute phase reactants (CRP, ESR) and D-dimer in patients with CTD-ILDcan be used as nonspecific inflammation activity indexes, during the treatment course,we can reexamine the above indicators, which is indicative to the disease progress, thetreatment effect and the withdrawal time of drugs. Detection of auto-antibodies isindicative to the disease development and prognosis except for the assistant functionin the diagnosis of CTD.