Severe Pulmonary Hypertension Due to Adult-Onset Still's Disease

Journal of Investigative Medicine High Impact Case Reports
Ankur SinhaKabu Chawla

Abstract

A 29-year-old female with adult-onset Still's disease (AOSD) presented with progressive shortness of breath both on rest and on exertion, increased abdominal girth, and swelling in both legs. She was on oral prednisone and was recently started on canakinumab (interleukin-1 antagonist) for joint pain and rash of AOSD. Echocardiogram showed severely dilated right ventricle, dilated pulmonary artery, moderately reduced right ventricular systolic function, but with normal left ventricular systolic function. Computed tomography with contrast ruled out pulmonary embolism. Blood tests ruled out other rheumatologic diseases. The patient was diagnosed with right-sided heart failure likely secondary to AOSD. Right heart catheterization was needed but could not be performed because of severely dilated pulmonary artery. The patient was transferred to a higher center for further management and possible cardiopulmonary transplant.

References

May 1, 1992·Annals of the Rheumatic Diseases·M Schwarz-EywillA Pezzutto
Jan 5, 2012·Chest·Laura C PriceMarc Humbert
Sep 14, 2012·Case Reports in Rheumatology·Marc Campos, Elena Schiopu
Dec 21, 2013·Journal of the American College of Cardiology·Gerald SimonneauRogiero Souza
Jan 18, 2014·Clinical Rheumatology·Petros EfthimiouBella Mehta

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Citations

Oct 10, 2018·Frontiers in Pharmacology·Paola GalozziPaolo Sfriso

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Adult-Onset Still's Disease

Adult-onset Still's disease (AOSD) is a form of Still's disease, a rare systemic autoinflammatory disease characterized by the classic triad of persistent high spiking fevers, joint pain, and a distinctive salmon-colored bumpy rash. Discover the latest research on AOSD here.

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