Pulmonary arterial hypertension in adult-onset Still's disease: A case series and systematic review of the literature

Narváez, Javier; Mora-Limiñana, Maribel; Ros, Inmaculada; Ibañez, Mónica; Valldeperas, Joan; Cremer, David De; Nolla, Joan M.; Juan-Mas, Antonio

doi:10.1016/j.semarthrit.2018.11.007

Cited by 16 publications

(13 citation statements)

References 45 publications

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“…Second-line therapies include methotrexate and biotherapies (interleukin-1 and -6 antagonists). Acute endocarditis with valvular vegetations due to fibrinoid or fibrinocruoric components has been reported, particularly involving the tricuspid valve ( 8 , 9 ).…”

Section: Discussionmentioning

confidence: 99%

“…A rare complication in AOSD is group 1 pulmonary arterial hypertension ( 9 ), which is more common in women, expressing as dry cough and with progressive dyspnea. Treatment consists of vasodilators (endothelin antagonists, prostacyclin agonists, nitric oxide agonists) used in pulmonary arterial hypertension in association with biotherapies.…”

Section: Discussionmentioning

confidence: 99%

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Acute Myocarditis Revealing Adult-Onset Still’s Disease

et al. 2021

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A 34-year-old man presented with fever, palpitations, maculopapular rash, pharyngitis, left cheilitis, and bilateral gonalgia. High-sensitivity troponin I concentration was 4,900 ng/l. Transthoracic echocardiogram revealed reduced global longitudinal strain. Cardiac magnetic resonance imaging showed acute myocarditis. Adult-onset Still’s disease was diagnosed, and treatment with intravenous corticosteroids and tocilizumab was initiated. ( Level of Difficulty: Beginner. )

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Acute Myocarditis Revealing Adult-Onset Still’s Disease

et al. 2021

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“…Only 21 case reports have described PAH complicated with AOSD. PAH is a rare complication of AOSD [2,3]. Connective tissue diseases (CTDs), such as systemic lupus erythematosus (SLE), Sjogren's syndrome (SjS), mixed CTD (MCTD), and systemic sclerosis (SSc), are more likely to complicate PAH.…”

Section: Discussionmentioning

confidence: 99%

Pulmonary artery hypertension prior to the relapse of adult‐onset Still's disease

Hara

Morita

Tanaka

et al. 2021

Respirology Case Reports

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Adult-onset Still's disease (AOSD) is a rare inflammatory autoimmune disorder characterized by fever, skin rash, and arthralgia. Pulmonary artery hypertension (PAH) rarely occurs with AOSD and has not been reported in the absence of typical symptoms of AOSD. A 33-year-old woman was admitted to our hospital with dyspnoea on exertion. Although she had not had symptoms of AOSD for 18 months before her admission, she presented with gradually progressing PAH. Because she had no typical symptoms of AOSD, she was treated with pulmonary vasodilators. However, her PAH did not improve. At one month after vasodilator treatment, she developed a high fever with elevation of ferritin. We determined that her AOSD had relapsed. Immunosuppressants were started and both her AOSD and PAH quickly improved. PAH may develop in the absence of typical symptoms of AOSD and immunosuppressants may be effective in such a case.

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“…Although primary endpoints were not met in the phase II faSScinate [ 223 ] and phase III focuSSced trials conducted in patients with SSc-ILD, there was some evidence of reduced lung function decline with the anti-IL-6R agent tocilizumab. In addition, case series have also reported modest effects in patients with SSc-ILD, with approximately 50% of patients achieving an improvement or stabilization of pulmonary function [ 224 ]. However, to date there is limited information on the effects of IL-6 blockade on ILD in patients with RA, with evidence limited to case reports describing anecdotal benefits regarding the off-label use of tocilizumab in patients with RA and ILD [ 225 – 227 ].…”

Section: Il-6 Beyond the Joint: Common Comorbidities Of Ramentioning

confidence: 99%

Understanding the Role of Interleukin-6 (IL-6) in the Joint and Beyond: A Comprehensive Review of IL-6 Inhibition for the Management of Rheumatoid Arthritis

Favalli

2020

Rheumatol Ther

101

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Rheumatoid arthritis (RA) is a chronic, debilitating autoimmune disorder involving inflammation and progressive destruction of the joints, affecting up to 1% of the population. The majority of patients with RA have one or more comorbid conditions, the most common being cardiovascular disease, osteoporosis, and depression, the presence of which are associated with poorer clinical outcomes and lower healthrelated quality of life. RA pathogenesis is driven by a complex network of proinflammatory cells and cytokines, and of these, interleukin-6 (IL-6) plays a key role in the chronic inflammation associated with RA. Through cell signaling that can be initiated by both membrane-bound and soluble forms of its receptor, IL-6 acts both locally to promote joint inflammation and destruction, and in the circulation to mediate extra-articular manifestations of RA, including pain, fatigue, morning stiffness, anemia, and weight loss. This narrative review describes the role of IL-6 in the pathogenesis of RA, its comorbidities, and extra-articular systemic manifestations, and examines the effects of the IL-6 receptor inhibitors sarilumab and tocilizumab on clinical endpoints of RA, patient-reported outcomes, and common comorbidities and extra-articular manifestations.

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Pulmonary arterial hypertension in adult-onset Still's disease: A case series and systematic review of the literature

Cited by 16 publications

References 45 publications

Acute Myocarditis Revealing Adult-Onset Still’s Disease

Acute Myocarditis Revealing Adult-Onset Still’s Disease

Pulmonary artery hypertension prior to the relapse of adult‐onset Still's disease

Understanding the Role of Interleukin-6 (IL-6) in the Joint and Beyond: A Comprehensive Review of IL-6 Inhibition for the Management of Rheumatoid Arthritis

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