A histologic evaluation of mixed connective tissue disease in childhood

Singsen, Bernhard H.; Swanson, Virginia L.; Bernstein, Bram; Heuser, Eva; Hanson, Virgil; Landing, Benjamin H.

doi:10.1016/0002-9343(80)90258-2

Cited by 77 publications

(20 citation statements)

References 27 publications

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“…It is also unlikely that age or obesity affects our results, because we could find no significant correlation between small airway dysfunction and age or body weight in the present study. However, pulmonary edema related to pulmonary inflammation may play a role in the production of uneven alveolar ventilation, since pulmonary edema has been reported for patients with MCTD (Cryer and Kissane 1978; Singsen et al 1980). Not all of the values of C 1 /Cst, V5o /V25 ratio, CV%, and RV%, which are parameters used to detect changes in small airways (McFadden et al 1974), were abnormal in a given patient.…”

Section: Resultsmentioning

confidence: 99%

Small Airway Involvement in Mixed Connective Tissue Disease.

Izumiyama

Hida

Ichinose

et al. 1993

Tohoku J. Exp. Med.

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We studied pulmonary functions in 17 female patients with mixed connective tissue disease (MCTD) to detect early pulmonary involvement in this disease; in 8 of the 17 patients followup studies were also performed at 1.2-to 5.9-year intervals. In the first pulmonary function tests, decreases in vital capacity (VC) and diffusing capacity (DL~o) were observed in 6 (35%) and 8 (47%) patients, respectively. The ratio of forced expiratory volume in one second to VC was normal in all the patients, but pulmonary resistance and static compliance were abnormal in 6 (35%) and 10 (59%) patients, respectively. However, frequency dependence of dynamic compliance was found in all 16 patients tested. Moreover, 4 (24%) of the 17 patients had normal DL~o and DL~o-to-alveolar volume ratio (DLCO/VA). Reductions in DL~o and DLCo/VA were significantly correlated with the disease duration. These results suggest that small airway obstruction is an early and frequent indication of functional pulmonary impairment, and that impairment of alveolar gas exchange is progressive in patients with MCTD.connective tissue disease; small airways; frequency dependence of dynamic compliance; diffusing capacity

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Section: Resultsmentioning

confidence: 99%

Small Airway Involvement in Mixed Connective Tissue Disease.

Izumiyama

Hida

Ichinose

et al. 1993

Tohoku J. Exp. Med.

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“…Cytokines are suggested to be intimately involved in the pathogenesis of proliferative vaseulopathy [11]. leading to pulmonary hypertension, a life-threatening eomplieation in MCTD.…”

Section: Discussionmentioning

confidence: 99%

Enhanced synthesis of cytokines by peripheral blood monocytes cultured in the presence of autoantibodies against U1-ribonucleoprotein and/or negatively charged molecules: implication in the pathogenesis of pulmonary hypertension in mixed connective tissue disease (MCTD)

Okawa-Takatsuji

Aotsuka

Uwatoko

et al. 1994

Clinical and Experimental Immunology

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SUMMARY An attempt was made to determine whether addition of purified autoantibodies against Ulribonucleoprotein (RNP) and negatively charged molecules (cardiolipin and double‐stranded (ds) DNA) to cultures of peripheral blood monoeytes could enhance the synthesis of eytokines in patients with MCTD and normal healthy volunteers. It was found that: (i) at the baseline, levels of cytokines such as IL‐1α, IL‐β and IL‐6 extracellularly released by or associated with monocytes were significantly higher in MCTD patients than in normal subjects; (ii) addition of antibodies against U1‐RNP to cultures of MCTD monocytes resulted in a significant overall increase of the released and cell‐associated IL‐1α, IL‐β and IL‐6. On the other hand, addition of antibodies against cardiolipin or dsDNA to cultures of MCTD monocytes resulted in a significant increase of released and/or cell‐associated IL‐1α and IL‐1β; (iii) addition of these autoantibodies to cultures of normal monocytes resulted in a significant overall increase of released and cell‐associated IL‐1α, IL‐1β and IL‐6. The extent of enhancement of cytokines released by or associated with monocytes was greater in normal subjects than in MCTD patients; (iv) a F(ab′)2 preparation of autoantibodies against U1‐RNP also enhanced the level of released and cell‐associated IL‐1α. Our findings that both autoantibodies against U1‐RNP and negatively charged molecules were able to enhance the synthesis of cytokines by monocytes suggest that these autoantibodies might cause derangement of endothelial cells and lead to proliferative vaseulopathy, which is a characteristic of pulmonary hypertension in MCTD.

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“…A bland intimal proliferation and medial hypertrophy affecting medium-sized and small vessels are the characteristic vascular lesions of MCTD (40,41). These changes have been reported in the lung, constituting the major cause of PH, and in the kidneys and myocardium (1 8,40,41).…”

Section: Cardiovascular Involvementmentioning

confidence: 92%

“…It should be suspected in any patient who has unexplained tachycardia, a loud pulmonic component of the second heart sound, or a gallop rhythm (18,37,38). Myocardial involvement can be due to inflammatory cell infiltrate and/or proliferative vasculopathy of the epicardial and intramural coronary arteries (18,38,40,41).…”

Section: Cardiovascular Involvementmentioning

confidence: 99%