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SHR Neuro Cancer Cardio Lipid Metab Microb

Olschewski, H.
Pulmonary hypertension and rheumatic diseases
PNEUMOLOGE. 2018; 15(6): 396-403. Doi: 10.1007/s10405-018-0195-5 [OPEN ACCESS]
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Leading authors Med Uni Graz: Olschewski Horst
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Abstract:: After idiopathic pulmonary arterial hypertension (IPAH), connective tissue disease (CTD)-associated PAH represents the largest subgroup within the PAH group of diseases. Systemic sclerosis (SSc) and mixed connective tissue diseases are at the highest risk of PAH; however, systemic lupus erythematosus (SLE), dermatomyositis, Sjogren's syndrome, antisynthetase syndrome and rheumatoid arthritis also show an increased risk for PAH. The pulmonary symptoms and treatment are similar to those for IPAH. For SSc ayearly echocardiographic screening for PAH is recommended. In the other CTDs this diagnostic measure is recommended only if signs and symptoms of PAH occur, particularly dyspnea on exertion, fatigue or fainting/syncope. An early therapy with targeted PAH medication is recommended, as the prognosis of CTD-associated PAH is much worse compared to IPAH. A CTD-associated pulmonary veno-occlusive disease (PVOD) must be considered as adifferential diagnosis, because this disease is particularly prone to treatment-associated side effects. Approved for targeted collagen-associated PAH are endothelin receptor antagonists (ERA), phosphodiesterase 5inhibitors (PDE5i), IP receptor agonists (IPA) and stimulators of soluble guanylate cyclase (sGCS). When combined with immunosuppressants and because of the special properties of the underlying diseases, targeted PAH medications are associated with higher risks of severe side effects compared to IPAH patients.
Find related publications in this database (Keywords): Collagen diseases; Connective tissue diseases; Idiopathic pulmonary arterial hypertension; Pulmonary veno-occlusive disease; Diagnostic techniques