ArticlePulmonary circulation2016
An atypical presentation of adult-onset Still's disease complicated by pulmonary hypertension and macrophage activation syndrome treated with immunosuppression: a case-based review of the literature.
Article in Pulmonary circulation, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers, 1 of them a synthesis that pooled it.
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Who cites it
12 citing papers in PubMed, 1 synthesis or guideline pooled it, 24 citations in OpenAlex.
- Management of adult-onset Still's disease with interleukin-1 inhibitors: evidence- and consensus-based statements by a panel of Italian experts.Arthritis research & therapy · 2019Pooled it
- The critical role of NFAT transcription factors in pulmonary arterial remodeling and pulmonary hypertension: from PASMC proliferation to therapeutic targeting.Molecular biology reports · 2026Review
- IL-1 Family Blockade in Cytokine Storm Syndromes.Advances in experimental medicine and biology · 2024Review
- Macrophage Activation Syndrome in the Setting of Rheumatic Diseases.Advances in experimental medicine and biology · 2024Review
- Strategy and Challenges of Paraclinical Examinations in Adult-Onset Still's Disease.Journal of clinical medicine · 2022Review
- Adult-Onset Still's Disease: Clinical Aspects and Therapeutic Approach.Journal of clinical medicine · 2021Review
- Adult-onset Still's disease evolving with multiple organ failure and death: A case report and review of the literature.World journal of clinical cases · 2021Article
- Macrophage activation syndrome and pulmonary arterial hypertension in a patient with adult-onset Still disease: A case report.Medicine · 2019Article
- Mechanisms, biomarkers and targets for adult-onset Still's disease.Nature reviews. Rheumatology · 2018Review
- Anakinra in Still's disease: a profile of its use.Drugs & therapy perspectives : for rational drug selection and use · 2018Article
- Adult-Onset Still's Disease: Still a Serious Health Problem (a Case Report and Literature Review).The American journal of case reports · 2017Review
- Pulmonary arterial hypertension secondary to adult-onset Still's disease: Response to cyclosporine and sildenafil over 15 years of follow-up.Respiratory medicine case reports · 2016Article
Corrections and comments
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Authors and funding
4 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Pulmonary arterial hypertension (PAH) is a known complication of rheumatologic diseases, but it is only rarely associated with adult-onset Still's disease (AOSD). We describe the case of a 30-year-old woman who presented in a pulmonary hypertension crisis and was found to have underlying AOSD with PAH and nonspecific interstitial pneumonia (NSIP) with a course complicated by macrophage activation syndrome (MAS). She dramatically improved with steroids, cyclosporine A, and anakinra, with total resolution of the MAS and significant improvement of her pulmonary arterial pressures. While there are only select case reports of AOSD associated with PAH, this is the first reported case of (1) AOSD complicated by both PAH and MAS and (2) AOSD complicated by biopsy-proven NSIP. Clinically, this case highlights the efficacy of immunosuppressive agents in the treatment of PAH and MAS from underlying AOSD and supports their use in this setting.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.