Interstitial lung disease in mixed connective tissue disease

INTRODUCTION: The authors analyzed the incidence of interstitial lung disease in mixed connective tissue disease. They were seeking an answer to the following problems: the nature of the pathological course of mixed connective tissue disease complicated by and the therapy to be used in interstitial lung disease. PATIENTS AND METHODS: 179 patients were followed up during a period of 15.9 +/- 6.1 years. Interstitial lung disease was diagnosed using high resolution computed tomography. The diagnosis of interstitial lung disease was not obvious in 5 patients thus open lung biopsy was performed, which confirmed common interstitial pneumonitis. The patients were followed-up, and the data of computed tomography and respiratory function tests were detected 6 months, and then 4 years after the acute lung disease complicated by mixed connective tissue disease. RESULTS: Out of the 179 mixed connective tissue disease patients 96 (53.6%) had interstitial lung disease. The onset of interstitial lung disease was the most frequent in the 2-4 years of the disease. Four years after the first appearance of interstitial lung disease severe fibrosis was diagnosed in 24 patients (25%). A honey comb formation in the lung developed only in one patient. For the treatment of interstitial lung disease, corticosteroid treatment had to be combined with cyclophosphamide in 51 cases. In 4 patients (24%), pulmonary arterial hypertension evolved 2-4 years following interstitial lung disease. The high pulmonary arterial pressure decreased using pulsed corticosteroid treatment, cyclophosphamide, prostacyclin analogue, anticoagulants therapy and the 4 patients stay alive. The pulmonary arterial hypertension was caused by obliterative vasculopathy. CONCLUSION: Pulmonary involvement is found in more than half of the patients with mixed connective tissue disease. Early diagnosis of interstitial lung disease is possible by computed tomography. Interstitial lung disease can be treated by the combination of corticosteroids and cyclophosphamide. The authors were the first to detect the coexistence of interstitial lung disease and pulmonary arterial hypertension in mixed connective tissue disease. Subsequent respiratory alterations in these patient necessitate regular patient follow up.     

Clinical observations in mixed connective tissue disease

The authors report their observations gathered by follow-up studies of many years duration on 67 patients suffering from mixed connective tissue disease. The period preceding the overt disease, its duration and its characteristic symptoms were examined. Concerning the severity of the clinical course, the authors distinguished between a less and a more severe form of the disease. Organ specific manifestations occurring in the two types of the disorder, the number of active phases per year, therapeutic tools and the prognosis were analysed. The authors assume that the more severe form of the disease may have some traits resembling those of systemic lupus erythematosus, but in the meantime, in the less severe type of the disease some features shared by rheumatic disorders can be found.     

Primary hemostasis in mixed connective tissue disease]

Willebrand-factor antigen level and structure analysis, ristomycin-cofactor assay, beta-thromboglobulin and thromboxane metabolite estimations were performed in 22 patients with mixed connective tissue disease. High levels of Willebrand factor antigen and activity were detected in the presence of thrombocytopenia, previous thrombotic events, pulmonary vascular lesions and in the presence of circulating antiendothelial antibodies. Increased platelet activation was documented also in antibody positive cases and in thrombocytopenia. The alterations of endothelial and platelet functions may play important role in the development of vascular complications of mixed connective tissue disease.     

Evaluation of survival in mixed connective tissue disease (MCTD)

INTRODUCTION: 179 patients with mixed connective tissue disease (MCTD) were follow-up, and the cause of death was analyzed in 12 died patients. PATIENTS AND METHODS: The survival of 179 patients with MCTD was evaluated by using Kaplan-Meier's method. Clinically and immunological data of the patients were analyzed between 1 and 25 years follow-up period (mean: 13.1 +/- 5.5 years). RESULTS: The five-year survival rate was 96.4%, 10-year survival rate was 93.9%, and the 15-year survival rate was 89.6%. The cause of death was pulmonary hypertension in 5 patients, thrombotic thrombocytopenic purpura/hemolytic uremic syndrome in 3 cases, infection in 3 cases (hepatitis C virus induced hepatic coma in 2 patients, Staphylococcus sepsis in one patient), and on one patient myocarditis. The pulmonary hypertension was the most serious prognostic factor. CONCLUSION: In patients with MCTD the pulmonary hypertension with endothelial cells proliferation and microangiopathy developed very quickly, and there was progressive and therapy resistant statement. The secondary virus and bacterial infections may develop in the patients who were followed-up the long term period. Their survival rate was better than the data in the literature. This fact may cause genetic-demographic factors, and the sequential follow-up of the patients.     

弥漫性结缔组织病相关不良妊娠的诊疗体会

<正>弥漫性结缔组织病是一种临床表现多样的自身免疫病,患者往往有多种自身抗体阳性,合并多组织、多器官受累,常见的如类风湿关节炎(rheumatoid arthritis,RA)、系统性红斑狼疮(systemic lupus erythematosus,SLE)和抗磷脂综合征(antiphospholipid syndrome,APS)等。因这类疾病好发于育龄期女性,故其与妊娠的关系备受关注。近十多年来,在我们的临床工作中,不断地出现弥漫性结缔组织病相关不良妊娠患者,诊疗中既有成功,也有失败,现总结体会如下。     

Cold lymphocytotoxins in connective tissue disorders.

Sixty-eight patients with various connective tissue disorders, 5 relatives of patients and 26 members of staff from the Centre for Rheumatic Diseases were studied for the presence in their sera of cold lymphocytotoxic antibodies. Antibodies were found in 71 percent of patients with systemic lupus erythematosus, 27 per cent of patients with rheumatoid arthritis, 0 per cent of the small group of relatives and 3.8 per cent of the controls. Absorption studies did not show T or B specificity of the antibodies. The control group, working in close proximity to the patients or their sera did not show any increased incidence of antibodies as compared to control groups of other studies. Red blood cell anti I or HI was found in the sera of 28 per cent of those with cold lymphocytotoxic antibodies. No correlation was found between the presence of the antibodies and number of blood transfusions or pregnancies, increasing age, R3 titre or antinuclear factor.     

The respiratory system in connective tissue diseases

Adolescents with connective tissue diseases (CTDs) are at risk for community-acquired and opportunistic respiratory tract infections; it is mandatory to consider an infectious etiology in the differential diagnosis. The authors highlight the respiratory manifestations of the more common CTDs seen in adolescence, and also discuss useful diagnostic tests and drug regimens.