The frequency of anti-beta2-glycoprotein I antibodies is low and these antibodies are associated with pulmonary hypertension in mixed connective tissue disease.

The objective of this study is to evaluate the prevalence of antiphospholipid antibodies, mainly anti-beta(2)-glycoprotein I (anti-beta(2)-GPI), and their possible clinical and laboratory relevance in mixed connective tissue disease (MCTD). This study included 39 consecutive patients with MCTD (Kasukawa's criteria) from January, 2005, to March, 2007, and compared them with 21 age- and sex-matched healthy controls. IgG and IgM anticardiolipin (aCL) and anti-beta(2)-GPI were measured by ELISA. Lupus anticoagulant (LA) was detected by functional coagulation tests. Medium to high titres of aCL and anti-beta(2)-GPI antibodies were found in sera from four (10.2%) MCTD patients. One of these patients was found to be positive for IgM aCL, IgM anti-beta(2)-GPI and LA antibodies simultaneously. Additionally, this patient had a previous history of foetal loss in the second trimester and new-onset pulmonary arterial hypertension (PAH). The other three patients had none of the manifestations of antiphospholipid syndrome (APS) or PAH. The mean value of IgG anti-beta(2)-GPI was higher among those MCTD patients with PAH than in the group without PAH (34.2 +/- 46.8 vs 12.3 +/- 9.1, P = 0.018). None of the controls were positive for antiphospholipid antibodies. High to moderate titres of anti-beta(2)-GPI as well as APS were rare in MCTD, and these antibodies may be correlated with the development of PAH in these patients.

Esophageal involvement and interstitial lung disease in mixed connective tissue disease.

RATIONALE: Mixed connective tissue disease is a systemic inflammatory disorder that results in both pulmonary and esophageal manifestations. OBJECTIVES: We sought to evaluate the relationship between esophageal dysfunction and interstitial lung disease in patients with mixed connective tissue disease. METHODS: We correlated the pulmonary function data and the high-resolution computed tomography findings of interstitial lung disease with the results of esophageal evaluation in manometry, 24-hour intraesophageal pH measurements, and the presence of esophageal dilatation on computed tomography scan. MEASUREMENTS AND MAIN RESULTS: Fifty consecutive patients with mixed connective tissue disease, according to Kasukawa's classification criteria, were included in this prospective study. High-resolution computed tomography parenchymal abnormalities were present in 39 of 50 patients. Esophageal dilatation, gastroesophageal reflux, and esophageal motor impairment were also very prevalent (28 of 50, 18 of 36, and 30 of 36, respectively). The presence of interstitial lung disease on computed tomography was significantly higher among patients with esophageal dilatation (92% vs. 45%; p<0.01) and among patients with severe motor dysfunction (90% vs. 35%; p<0.001). CONCLUSIONS: Although we were not able to prove a causal relationship between esophageal and pulmonary involvement, our series revealed a strong association between esophageal motor dysfunction and interstitial lung disease in patients with mixed connective tissue disease.

Radionuclide imaging for the assessment of esophageal motility disorders in mixed connective tissue disease patients: relation to pulmonary impairment.

Esophageal functional abnormalities may lead to regurgitation, chronic esophagitis and life-threatening conditions such as aspiration pneumonia. In mixed connective tissue disease patients, previous reports showed that esophageal dysfunction varies according to the method employed for investigation. Our study was conceived to: (i) assess esophageal motility and mucosal aspects in patients with mixed connective tissue disease by endoscopy, cine-esophogram and scintigraphy focusing on the prevalence of each exam; and (ii) verify the association between pulmonary and esophageal dysfunctions. Twenty-four mixed connective tissue disease patients were enrolled for this study. Cine-esophogram and upper digestive endoscopy with mucosal biopsy were performed according to previous standardization. Radionuclide esophageal scintigraphy was performed with a semisolid meal with (99m)Tc. Eleven healthy individuals voluntarily submitted to scintigraphy as controls. Cine-esophogram showed esophageal delayed emptying in 90% of patients. At scintigraphy there was a significant delay in total esophageal transit time in the group of patients when compared to healthy controls (35.3 +/- 8.2 s. vs. 13.6 +/- 9.5 s.; P < 0.0001). The whole esophageal body showed dysmotility in 96% of patients. The cine-esophogram detected functional esophageal impairment similar to scintigraphic findings. Histopathologic examination found esophagitis in 95% of studied patients. Reduced lung volumes were associated with esophagitis and delayed esophageal clearance at scintigraphy, observed at the distal portion of the esophagus. Esophageal scintigraphy is easy to perform, with good acceptance by patients with low radiation exposition. It is a useful non-invasive test for follow-up and interventional studies concerning esophagus dysfunction.