[A case of systemic lupus erythematosus complicated with pulmonary hypertension and massive pericardial effusion]

A 22 year-old-female had suffered from polyarthralgia and Raynaud's phenomenon since 1984. In 1986, she was diagnosed as systemic lupus erythematosus (SLE). In April 1988, she was admitted to Kawasaki Municipal Hospital because of fever and dyspnea on exertion (DOE). Physical examination showed high fever, butterfly rash, oral ulcer and elevation of heart sound IIp on auscultation. Laboratory findings revealed that erythrocyte sedimentation rate was elevated to 105 mm/hr. The following values were observed, anti DNA antibody 391 IU/ml, serum IgA 5mg/dl, anti IgA antibody weakly positive. Chest X ray showed CTR 65%. Echo cardiogram showed massive pericardial effusion. 201T1 myocardial SPECT revealed right ventricular pressure over loading. PSL 40 mg/day was started to administer for the massive pericardial effusion due to SLE activities. On 6th of June, right heart catheterization confirmed the pulmonary hypertension (PPA 22 mmHg, Pulmonary artery resistance (PAR) 1163 dyne/sec/cm-5/mm2). By the treatment with PSL, massive pericardial effusion was gradually improved but DOE clinically unchanged. Second right heart catheterization was done on 8th of August. PAR was improved to 895 dyne/sec/cm-5/mm2 but PPA was elevated to 26 mmHg. It is very interesting that PPA was elevated although PAR was improved by PSL therapy. It is considered that the increase in venous return which was caused by improvement of massive pericardial effusion induced conversely the elevation of PPA. Additionally she was complicated with IgA deficiency. It may occur not only by the immunogenetical disorder such as HLA or IgG subclass alteration but also by anti IgA antibody or lymphocytes dysfunction complicated with SLE.