Pulmonary vascular lesions in mixed connective tissue disease

Pulmonary vascular lesions in MCTD.

(Panel A) Severe intimal fibrosis almost occluding the vascular lumen (Elasitica-Goldner stain).

(Panel B) Organized thrombus in medium-sized artery (Elasitica-Goldner stain).

(Panel C) Plexogenic arteriopathy sometimes occurred (Elasitica-Goldner stain).

(Panel D) Vasculitis of small vessels with inflammatory infiltration around vessels almost composed of lymphocytes (Elastica-Goldner stain).

MCTD cases with PH also show the interstitial type, vascular type, and mixed type with both lesions; however, the mixed type is very rare. Further, the vascular type of MCTD shows similar lesions to SSc, such as fibrous thickening, and to SLE, such as plexogenic arteriopathy.