Pulmonary arterial hypertension complicating adult-onset Still's disease.
Adult-onset Still's disease (AOSD) is a rare condition diagnosed by a combination of clinical and laboratory features and after ruling out other conditions. Pulmonary manifestations, apart from pleuritis, are uncommon and pulmonary arterial hypertension (PAH) in extremely uncommon. We have described a case of AOSD with severe PAH. There have been rare reports of PAH occurring in AOSD in the literature. Probably, this manifestation may have been understudied, being confined to cases which are symptomatic. A larger study to look at the asymptomatic occurrences of PAH in AOSD may help in unraveling the mystery of this disease.
Thakare
Manisha
M
Department of Rheumatology, Nizam's Institute of Medical Sciences, Punjagutta, Hyderabad, India.
Habibi
Shabina
S
Agrawal
Sumeet
S
Narsimulu
Gumdal
G
D000293:Adolescent; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology; D035583:Rare Diseases; D016706:Still's Disease, Adult-Onset / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology
D000894:Anti-Inflammatory Agents, Non-Steroidal; D005938:Glucocorticoids