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Interstitial lung disease (ILD) is a frequent complication of patients with connective tissue disease (CTD) and significantly affects morbidity and mortality. Disease course may vary from stable or mildly progressive to more severe, with rapid loss of lung function. At present, there are great challenges and poor prognosis in the diagnosis and treatment of CTD-ILD. Based on the evidence and guidelines from China and other countries, experts from the Chinese Rheumatology Association developed standardization of diagnosis and treatment of CTD-ILD. The aim is to strengthen the early identification of, standardize the diagnosis and treatment of CTD-ILD, and delay the progress of the disease.
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Objective To analyze the relationship between ferritin, carbohydrate antigen (CA)125, CA153, lactic dehydrogenase (LDH), albumin and progression and prognosis of Sj?gren's syndrome with inter-stitial pneumonitis (pSS-IP). Methods Five hundred and twenty-seven primary pSS patients from 2011 to 2017 were analyzed retro-spectively. According to the lung imaging and clinical features, they were divided into groups. The differences of ferritin, CA125, CA153, LDH and albumin (ALB) levels in each group were analyzed by Mann-Whitney U test. Logistic regression was used to analyze the risk factors of pSS complicated with interstitial pneumonitis. Results ① Among the 527 pSS patients, 206 (39.1％) were diagnosed with IP. Compared with 321 patients without interstitial pneumonitis (pSS-N-IP), ferritin, CA125, CA153 and LDH in pSS-IP were relatively higher [189(116, 380) ng/ml vs 138(75, 258) ng/ml, Z=-3.777, P<0.05;19(12, 36) U/ml vs 12(9, 19) U/ml, Z=-5.974, P<0.05;22(12, 34) U/ml vs 9(6, 14) U/ml, Z=-4.582, P<0.05;206(165, 258) U/L vs 161(139, 195) U/L, Z=-8.770, P<0.05], while albumin was lower [34(31, 37) g/L vs 36(34, 39) g/L, Z=5.210, P<0.05]. ② pSS-IP were divided into pSS-IP progression group (46 cases) and pSS-IP stable group (60 cases) according to their lung imaging characteristics. Compared with pSS-IP stabilization group, pSS-IP progression group had higher ferritin [254(129, 693) ng/ml vs 161(104, 259) ng/ml, Z=-2.437, P<0.05]. ③ Logistic regres-sion analysis showed that there was correlation between ferritin and the progress of pSS-IP (OR=1.002). Conclusion Ferritin, CA125, CA153, LDH, albumin are related to pSS-IP. Ferritin isrelated to the progress of pSS-IP, and is a risk factor for the progress of pSS-IP.
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Objective To analyze the clinical characteristics of patients with panniculitis who had breast involvement and to expand the understanding of the organ involvement of panniculitis.Methods The data of panniculitis patients with breast involvement were collected from our hospital during 2012.1.1 to 2017.7.1,including sex,age,clinical manifestations,laboratory tests,imaging examinations and treatments.Results A total of 7 panniculitis patients were included who had breast involvement.All patients were female,aged 28 to 45 years [mean,(35±6) years].Clinical manifestations included hypothermia (4/7),arthralgia (4/7),limb subcutaneous nodules (7/7) and breast nodules (7/7).Breast nodules occurred 1 to 4 months earlier than the limbs subcutaneous nodules in 6 cases and 11 years later than the limbs subcutaneous in 1 case.For laboratory tests:all patients had increased of erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) (7/7) while only 2 patients had elevation of white blood cell count (2/6).One patient had breast enhanced magnetic resonance imaging (MRI) scanning.Seven patients underwent biopsy of limb subcutaneous nodules.The pathological findings were all consistent with the pathological characteristics of panniculitis.Three cases had breast nodules biopsy,which showed inflammation and decrease of fat tissue,several kinds of cells infiltration and granuloma formation.Treatment with antibiotic failed while glucocorticoid and immunosuppressive agents treatment was effective.Conclusion Pancreatitis can have breast involvement,mostly in young women,with breast painful nodules in most patients early than limb subcutaneous nodules.Breast enhancement MRI scanning and nodules biopsy have important value in the diagnosis of breast panniculitis.For patients with breast nodules as the first symptom and fail to response to antibiotic treatment,clinicians need to be alert to breast panniculitis and perform pathological biopsy to reduce the chance of misdiagnosis.
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Objective To investigate the treatment compliance and uric acid control rate in patients with primary gout.Methods One hundred primary gout patients with disease duration ＞ 6 months treated in our hospital from June 2015 to June 2016 were enrolled in the study.The treatment compliance and disease control rate were evaluated with revised Chinese Compliance Questionnaire-Rheumatology (CCQR).Results Eighty four valid questionnaires was recovered for analysis.Among 84 patients there were 46 cases of high compliance (54.8％) and 38 cases of poor compliance (45.2％).The CCQR score in high compliance group was significantly higher than that in poor compliance group (73.48 ± 15.62 vs.48.37 ±10.96,t =8.352,P ＜ 0.001).There were no significant differences in education level,personal income,family history,health insurance and type of uric-acid-lowering drugs between high compliance group and low compliance group (P ＞ 0.05).The duration of gout was shorter in high compliance group than that in low compliance group [(6.12 ± 1.79) y vs.(13.24 ± 4.56) y,P ＜ 0.001].The uric acid control rate in high compliance group was higher than that in poor compliance group (76.1％ vs.36.8％,x2 =13.186,P =0.000 3);and the serum uric acid levels were (369.82 ± 35.67)μmol/L and (497.46 ± 63.49)μmol/L in two groups,respectively (t =11.606,P ＜ 0.001).Conclusion It is necessary to strengthen health education for gout patients to improve the treatment compliance and to achieve the high uric acid control rate.
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Objective To investigate the clinical data of a patient with IgG4-related disease involving the trachea and paratracheal soft tissue and review the literature so as to improve the understanding level of the disorder.Methods To analyze the clinical manifestation,laboratory examination,imaging,histopathology,treatment and prognosis of a patient with IgG4-related disease trachea and paratracheal soft tissue involved,who was admitted to the Department of Respiratory and Critical Care Medicine at Beijing Chaoyang Hospital.The relevant literatures were reviewed.Results A 18-year-old female was admitted with chief complaint of cough,dyspnea,and neck mass.Neck CT suggested that tracheal stenosis was caused by surrounded soft tissue.Paratracheal mass biopsy showed dense collagen fibers with infiltration of many lymphocytes and plasma cells.Immunohistochemical stain found that IgG4-positive plasma cells were ＞ 50/high power field (HPF) and a ratio of IgG4/IgG positive cells was over 40％.The level of serum IgG4 was significantly increased (2 930 mg/L).She was diagnosed as IgG4-related disease.The patient was treated with 80 mg intravenous methylprednisolone per day for three days,then prednisone 40 mg daily oral.Her dyspnea was significantly relieved.One month later,CT scan showed that the cervical tracheal stenosis was significantly improved.We identified 20 cases of IgG4-related disease involving the trachea and paratracheal soft tissue from databases,in which only 1 case was similar as this patient.The other 19 cases were of extratracheal involvement.Elevated serum IgG4 was detected in 11/12 patients.Most patients were treated with glucocorticoid,some combined with immunosuppressive agents and rituximab.The clinical outcome was good.Conclusion IgG4-related disease involving the trachea and paratracheal soft tissue is a rare condition.Serum IgG4 level and histopathology should be considered for diagnosis.Glucocorticoid is effective.
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Objective To study the characteristics of granulomatosis with polyangiitis (GPA) accompanied by pneumothorax.Methods We described a case of GPA accompanied by hydropneumothorax who was successfully treated.Relevant literature was also reviewed.Results A total of 25 cases were identified,consisting of 18 males and 7 females [the average age was (44±16)(16-70) years old].The time from disease onset to pneumothorax was 26±51 (0.83-216) weeks.Pneumothorax,hydropneumothorax,pyopneumothorax and hemopneumothorax occurred in 11,5,8 and 1 respectively.Nodules or excavated nodules on chest radiography or CT were seen in 22 cases.Erythrocyte sedimentation rate (ESR) and C reactive protein (CRP) were elevated in all cases.Sixteen cases received glucocorticoid and immunosuppressive agents treatment.Sixteen cases received drainage and 7 received open operation.Pseudomonas aeruginosa was the most commonmicrobiology findings.Granulomatosis with active vasculitis,bronchopleural fistula,pleural bleb with intensefibrosis,rupture of subpleural nodule were seen on lung biopsy or autopsy.Nine cases died of infections,respiratory failure,sepsis and respiratory arrest.Conclusion Pneumothorax in GPA can be caused by multiple factors such as rupture of subpleural nodule and with high mortality.Patients always died of infections and respiratory failure.Regular treatment of the underlying disease,apply sensitive antibiotics for infection and reasonable surgical intervention should be considered.
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Objective To investigate the manifestations of esophageal motility disorders and evaluate the association between them and dysphagia, laboratory tests and other accessory examinations in patients with Sj(o)gren's syndrome (SS). Methods Esophageal manometry was performed in 31 patients with SS and 18 healthy volunteers by the step pull-through method. Results Decreased upper esophageal sphincter pressure was detected in 19 of the 31 patients (61%) with SS, while 4 of 18 (22%) in controls. The frequency was significantly higher in patients than in healthy controls (P=0.008). Fifteen of 31 patients (48%) showed various patterns of esophageal dysfunction including ineffective esophageal motility in 6 patients, nutcracker esophagus in 3 patients and nonspecific dysmotility in 6 patients. No major differences were found in esophageal parameters (peak amplitude, wave duration and velocity) when comparing primary SS with secondary SS. These esophageal abnormalities were not correlated with clinical manifestations, laboratory examinations and other auxiliary examinations. Conclusion Patients with SS may have esophageal motility disorders, which can presents with different patterns.
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Objective To investigate the prevalence and clinical characteristics of Sj?gren′s syndrome-interstitial lung disease (SS-ILD). Methods 136 patients with SS were studied. Anti-SSA and Anti-SSB antibodies were measured by Western blot. The inpatients had chest X ray, chest HRCT and pulmonary function examined. Results ①pSS-ILD patients with postive anti-SSA antibody were proned to have interstitial lung disease and the ILD were more severe. ②HRCT showed that sSS-ILD were more severe than that of pSS-ILD. ③Lung capacity of pSS-ILD decreased more frequently than sSS-ILD. sSS-ILD mainly had venti-latory function abnormalities. The lung function impairment of both were dominated by small airways dysfunction and decrease of TLCO. Conclusion SS patients should be examined by HRCT and lung function tests should be performed in the course of the disease to find out and treat ILD.