sildenafil-citrate and Lupus-Erythematosus--Systemic

We report a rare case of diffuse systemic sclerosis (SSc) evolving into diffuse SSc/systemic lupus erythematosus (SLE) overlap syndrome. A 15-year-old boy was diagnosed as diffuse SSc with initial presentations of Raynaud's phenomenon and skin tightening. He underwent Chinese herbal treatment and clinical symptoms deteriorated in the following 3 years. On admission to our ward, serositis with pleural effusion, severe pulmonary fibrosis with moderate pulmonary hypertension, swallowing difficulty, and polyarthritis were observed. Autoantibody profiles revealed concurrence of anti-double-stranded DNA, anti-Smith, anti-topoisomerase I, and anti-ribonucleoprotein antibodies. The patient fulfills the criteria for both diffuse SSc and SLE. After drainage for pleural effusion accompanied by oral prednisolone and sildenafil, there were improvement of respiratory distress, swallowing difficulty, and pulmonary hypertension. In conclusion, connective tissue diseases may overlap with each other during the disease course. Serial follow-up for clinical symptoms as well as serological changes is recommended.

Topics: Adolescent; Antihypertensive Agents; Arthritis; Autoantibodies; Biomarkers; Deglutition Disorders; Disease Progression; Drainage; Drugs, Chinese Herbal; Glucocorticoids; Humans; Hypertension, Pulmonary; Lupus Erythematosus, Systemic; Male; Piperazines; Pleural Effusion; Prednisolone; Pulmonary Fibrosis; Purines; Raynaud Disease; Scleroderma, Diffuse; Serositis; Sildenafil Citrate; Sulfones; Treatment Outcome

Sildenafil and bosentan were added recently to the treatment with great expectations, effectiveness for the acute exacerbation of pulmonary arterial hypertension (PAH) is not fully examined. Two cases of acutely exacerbated PAH associated with collagen vascular diseases were treated first with sildenafil for six months followed by bosentan for another six months and the characteristics of this treatment modality were examined. Sildenafil showed an immediate effect which started in as early as approximately 30 min and was maximized in 60-90 min after oral ingestion. Continuous use of sildenafil for six months lowered pulmonary arterial pressure, pulmonary vascular resistance and the levels of brain natriuretic peptides along with an increased distance in 6-minute-walk, and replacement of it to with bosentan kept these effects. We think it as a treatment choice to use sildenafil first as a reliever and replace it with a controller bosentan, considering the immediate effects of sildenafil.

Topics: Adult; Antihypertensive Agents; Bosentan; Drug Therapy, Combination; Exercise Tolerance; Female; Humans; Hypertension, Pulmonary; Lupus Erythematosus, Systemic; Mixed Connective Tissue Disease; Natriuretic Peptide, Brain; Piperazines; Purines; Sildenafil Citrate; Sulfonamides; Sulfones; Vasodilator Agents

Topics: Adult; Anti-Inflammatory Agents; Cyclophosphamide; Drug Therapy, Combination; Dyspnea; Female; Humans; Hypertension, Pulmonary; Immunosuppressive Agents; Lupus Erythematosus, Systemic; Methylprednisolone; Piperazines; Purines; Sildenafil Citrate; Sulfones; Treatment Failure; Vasodilator Agents

A 46-year-old woman with systemic lupus erythematosus (SLE) and concomitant severe pulmonary hypertension (PH) is described. Other secondary causes of PH including thromboembolism, phospholipid syndrome, valvular disease and interstitial pulmonary involvement were ruled out. Owing to her lack of clinical response to conventional therapy, sildenafil was begun at increasing doses up to 400 mg daily. Both clinical and hemodynamic improvement ensued. This appears to be the first clinical report of the use of sildenafil in SLE followed by resolution of severe PH.

Topics: Administration, Oral; Female; Humans; Hypertension, Pulmonary; Lupus Erythematosus, Systemic; Middle Aged; Piperazines; Purines; Sildenafil Citrate; Sulfones; Treatment Outcome; Vasodilator Agents

Subcutaneous prostacyclin (treprostinil) is an effective short-term treatment for pulmonary hypertension. The most frequently described adverse effect-pain in the area of injection-rarely requires that treatment be withdrawn. Sildenafil is a selective fosfodiesterase-5 inhibitor with pulmonary vasodilating effects. We describe the use of sildenafil as a substitute for treprostinil in a patient with pulmonary hypertension associated with lupus erythematosus. Treatment with treprostinil was discontinued due to uncontrollable abdominal pain.

Topics: Abdominal Pain; Adult; Epoprostenol; Female; Humans; Hypertension, Pulmonary; Lupus Erythematosus, Systemic; Piperazines; Purines; Sildenafil Citrate; Sulfones; Vasodilator Agents