An expert opinion of the Polish Cardiac Society Working Group on Pulmonary Circulation and the Polish Society for Rheumatology on the diagnosis and treatment of pulmonary hypertension in patients with connective tissue disease

Mularek‐Kubzdela, Tatiana; Ciurzyński, Michał; Bielecka, Otylia Kowal; Kasprzak, Jarosław D.; Kopeć, Grzegorz; Mizia‐Stec, Katarzyna; Mroczek, Ewa; Lewicka, Ewa; Skoczylas, Ilona; Grabka, Marek; Furdal, Michał; Florczyk, Michał; Brzosko, Marek; Batko, Bogdan; Przepiera-Będzak, Hanna; Samborski, Włodzimierz; Kucharz, Eugene J.

doi:10.33963/kp.a2021.0055

Cited by 7 publications

(6 citation statements)

References 52 publications

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“…PAH-CTD is a progressive disease that must be detected early (patients with SSc should be screened annually), and composite drug therapy is recommended to be implemented as early as possible [ 73 ]. Despite a serious prognosis, PAH-CTD can be effectively treated with PAH-specific drugs.…”

Section: Discussionmentioning

confidence: 99%

Recent Advances in the Treatment of Pulmonary Arterial Hypertension Associated with Connective Tissue Diseases

Smukowska-Gorynia,

Gościniak,

Woźniak

et al. 2023

Pharmaceuticals

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Pulmonary hypertension (PH) is a severe vascular complication of connective tissue diseases (CTD). Patients with CTD may develop PH belonging to diverse groups: (1) pulmonary arterial hypertension (PAH), (2) PH due to left heart disease, (3) secondary PH due to lung disease and/or hypoxia and (4) chronic thromboembolic pulmonary hypertension (CTEPH). PAH most often develops in systemic scleroderma (SSc), mostly in its limited variant. PAH-CTD is a progressive disease characterized by poor prognosis. Therefore, early diagnosis should be established. A specific treatment for PAH-CTD is currently available and recommended: prostacyclin derivative (treprostinil, epoprostenol, iloprost, selexipag), nitric oxide and natriuretic pathway: stimulators of soluble guanylate cyclase (sGC: riociguat) and phosphodiesterase-five inhibitors (PDE5i: sildenafil, tadalafil), endothelin receptor antagonists (ERA: bosentan, macitentan, ambrisentan). Moreover, novel drugs, e.g., sotatercept, have been intensively investigated in clinical trials. We aim to review the literature on recent advances in the treatment strategy and prognosis of patients with PAH-CTD. In this manuscript, we discuss the mechanism of action of PAH-specific drugs and new agents and the latest research conducted on PAH-CTD patients.

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Section: Discussionmentioning

confidence: 99%

Recent Advances in the Treatment of Pulmonary Arterial Hypertension Associated with Connective Tissue Diseases

Smukowska-Gorynia,

Gościniak,

Woźniak

et al. 2023

Pharmaceuticals

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“…ЛАГ-ассоциированный фенотип ССД можно установить при анализе анамнестических сведений (длительность синдрома Рейно более 12 лет до первых признаков ЛАГ), клинических (телеангиэктазии, лимитированное поражение кожи или его отсутствие) и лабораторных (гиперурикемия, наличие антицентромерных антител, отсутствие антител к топоизомеразе-1) показателей, связанных с развитием ЛАГ при ССД [270].…”

Section: лёгочная артериальная гипертензия ассоциированная с системны...unclassified

Eurasian guidelines for the diagnosis and treatment of pulmonary hypertension (2023)

Chazova,

Martynyuk,

Shmalts

et al. 2024

Evrazijskij kardiologičeskij žurnal

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“…According to current European Society of Cardiology (ESC)/European Respiratory Society (ERS) guidelines, all asymptomatic patients with SSc and connective tissue diseases at risk of developing scleroderma should be monitored for the development of PAH. Any SSc patient with suspected pulmonary hypertension needs to have their indication determined for right heart catheterisation (RHC), which is the only examination to confirm the diagnosis of pulmonary hypertension [13].…”

Section: Pulmonary Arterial Hypertension (Pah)mentioning

confidence: 99%

Everything you always wanted to know about systemic sclerosis but were afraid to ask: Part 5. Treatment of patients with systemic sclerosis characteristics and treatment recommendations for interstitial lung disease, cardiac involvement, pulmonary arterial hypertension, musculoskeletal, gastrointestinal and renal involvement

Bultrowicz,

Kopeć-Mędrek,

Kopeć-Mędrek

et al. 2023

Rheumatology Forum

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Systemic sclerosis (SSc) is a systemic connective tissue disease characterised by disseminated microangiopathy and excessively immune-stimulated fibroblast activity, leading to fibrosis of the skin and internal organs. In the literature, the first report of the disease dates back to 1753 and is attributed to the physician Carlo Curzio of Naples, who described the case of a 17-year-old girl who developed induration of the skin all over her body. The disease is a rare condition, with an estimated 1 in 10 000 people in Poland suffering from SSC. Women predominate among patients, with a 3-4-fold advantage over men. The disease typically has its onset between the ages of 30 and 50. Early detection and treatment of organ complications are key to improving quality of life and reducing mortality in SSc patients. Given the considerable variability of the clinical course, it seems justified to take an individual approach to patients and cooperate with multiple specialists, both at the stage of diagnosis and at the stage of treatment. Treatment is based on what is known as an organ-specific strategy and consists of tailoring pharmacotherapy to the clinical presentation, disease period and organ complications. In addition to pharmacology, the treatment of patients should also include education of the patient and their family, and -if necessary -surgical treatment or other necessary interventions.

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An expert opinion of the Polish Cardiac Society Working Group on Pulmonary Circulation and the Polish Society for Rheumatology on the diagnosis and treatment of pulmonary hypertension in patients with connective tissue disease

Cited by 7 publications

References 52 publications

Recent Advances in the Treatment of Pulmonary Arterial Hypertension Associated with Connective Tissue Diseases

Recent Advances in the Treatment of Pulmonary Arterial Hypertension Associated with Connective Tissue Diseases

Eurasian guidelines for the diagnosis and treatment of pulmonary hypertension (2023)

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