In three placebo-controlled, double-blind studies, low-dose, full body UVA1 irradiation administered 2-3 times a week had a remedial action in patients with systemic lupus erythematosus (SLE) [1][2][3] . SLE Activity Measure (SLAM) and SLE Disease Activity Index (SLEDAI) scores decreased significantly. The therapy mitigated fatigue, joint pain, rashes, cognitive dysfunction, and photosensitivity within days to weeks, and over the years appeared to maintain these gains 4 . In only one of these studies was an effect on dyspnea recorded, 4/4 patients experiencing decreases 3 . Although the lungs are clearly a target in SLE, there has been no patient with pulmonary disease, prior to this report, monitored for changes in pulmonary measures during prolonged UVA1 irradiation therapy.
Case ReportA 39-year-old woman with SLE of five years duration presented with fatigue, a malar rash, polyarthritis, photosensitivity, dyspnea, interstitial lung disease (ILD) and pulmonary hypertension (PH). She had a history also of mouth ulcers, intermittent leukopenia, pericarditis, and recurrent pleurisy. She had an ANA of 1: 640, an IgM anticardiolipin antibody of 21 MPL U/mL (normal: 0-9), an increased anti-RNP, and prior to entering the study, elevated anti-SSA antibodies. Anti-dsDNA antibodies were absent and the sedimentation rate, CRP, and C3 were normal throughout the study. At the outset of the treatment, the C4 was low at 16 mg/dl (normals 18-45) and the WBC 2.7 (×10 3 ), but the C4 became normal and the WBC, ranged from 4-6 (×10 3 ) after the start of therapy. She had been taking 8 mg of methylprednisolone a day and 200 mg of hydroxychloroquine twice daily for over a year. The initiation of low-dose (8J/ cm 2 ) full body, twice weekly exposures to UVA1 irradiation, as previously described 1 , led to resolution of her fatigue and malar rash within days, polyarthritis and cognitive deficits within weeks, and photosensitivity within months, during which she discontinued her prednisone but continued the hydroxychloroquine. Although the study was not directed at interstitial lung disease (ILD) or pulmonary hypertension (PH), over the long term her pleurisy subsided, dyspnea diminished, restricted pulmonary volume increased, and the DLCO as measured by single-breath standard technique increased from 14 to 24 ml/min/mmHg ( Figure), representing a 65% to 105% increase of predicted. Pulmonary pressures, measured by trans-thoracic ultrasound, decreased from 45 to 25 mmHg. Her ANA remained at 1: 640, ENA and anticardiolipin antibodies became negative, and the anti dsDNA, sedimentation rate and CRP remained normal. She gradually discontinued her prednisone and progressed through a successful pregnancy while continuing the UVA1 therapy. Unfortunately, due to an unforeseen and permanent interruption of therapy, her condition deteriorated, the DLCO decreased to 17 mm/min/mmHg ( Figure) and the patient required resumption of corticosteroid therapy. Her lung volumes worsened with pulmonary function studies revealing a mild restrictive defect....