nintedanib and Scleroderma--Diffuse

You are consulted to evaluate a 56-year-old woman with known Raynaud's phenomenon, finger swelling of several; months' duration, and new hypertension with a blood pressure of 160/100 mm/Hg. She also reports progressive shortness of breath. Physical examination reveals telangiectasias, sclerodactyly, and proximal skin sclerosis (thick shiny skin on the chest and upper arms), and bibasilar crackles are found on chest examination. Laboratory tests reveal evidence of microangiopathic hemolytic anemia, thrombocytopenia, and elevation of the serum creatinine level (previously normal), and chest computed tomography shows evidence of ground-glass opacification in both lower lung fields.

Topics: Adrenergic alpha-Antagonists; Angiotensin Receptor Antagonists; Angiotensin-Converting Enzyme Inhibitors; Antibodies, Antinuclear; Antibodies, Monoclonal, Humanized; Antihypertensive Agents; Antirheumatic Agents; Calcium Channel Blockers; Complement C3; Complement C4; Complement Inactivating Agents; Cyclophosphamide; Endothelin Receptor Antagonists; Humans; Hypertension; Indoles; Lung Diseases, Interstitial; Plasma Exchange; Protein Kinase Inhibitors; Raynaud Disease; Renal Insufficiency; RNA Polymerase III; Scleroderma, Diffuse; Scleroderma, Limited; Stem Cell Transplantation; Tomography, X-Ray Computed; Transplantation, Autologous; Vasodilator Agents