Exercise and Respiratory Therapy in Patients With Rheumatoid Arthritis / Collagenosis and Pulmonary Hypertension

• Change in the 6-minute walking distance [ Time Frame: after 3 weeks and after 15 weeks compared to baseline. ] [ Designated as safety issue: Yes ]
• Quality of life (SF-36) [ Time Frame: baseline and 15 weeks ] [ Designated as safety issue: No ]

• Change in the 6-minute walking distance [ Time Frame: after 3 weeks and after 15 weeks compared to baseline. ]
• Quality of life (SF-36)

• change in inflammatory parameters (BKS, CRP, Leucocytes), change in inflammatory cytokines (TNF-α), change in parameters of the vascular endothelium (Endothelin, PDGF, VEGF), and endothelial progenitor cells [ Time Frame: baseline and 15 weeks ] [ Designated as safety issue: No ]
• Physical capacity in the cardiopulmonary exercise testing (Watt) [ Time Frame: baseline, 3 weeks, 15 weeks ] [ Designated as safety issue: Yes ]
• change of peak oxygen consumption and other parameters of cardiopulmonary exercise testing. [ Time Frame: baseline, 3 weeks, 15 weeks ] [ Designated as safety issue: Yes ]
• hemodynamic parameters: dimension and pump function of the right and the left ventricle. [ Time Frame: baseline, 15 weeks ] [ Designated as safety issue: Yes ]
• change in systolic pulmonary arterial pressure at rest and during exercise [ Time Frame: baseline, 3 weeks, 15 weeks ] [ Designated as safety issue: Yes ]
  echocardiography
• change of NTproBNP-value [ Time Frame: baseline, 3 weeks, 15 weeks ] [ Designated as safety issue: Yes ]

• change in inflammatory parameters (BKS, CRP, Leucocytes), change in inflammatory cytokines (TNF-α), change in parameters of the vascular endothelium (Endothelin, PDGF, VEGF), and endothelial progenitor cells
• Physical capacity in the cardiopulmonary exercise testing (Watt)
• change of peak oxygen consumption and other parameters of cardiopulmonary exercise testing.
• hemodynamic parameters: dimension and pump function of the right and the left ventricle.
• change in systolic pulmonary arterial pressure at rest and during exercise
• change in strength of respiratory muscles
• change of MRI-parameters
• change of NTproBNP-value

In Patients with rheumatic disease exercise training is a well established element of therapy. In contrast patients with severe pulmonary hypertension are advised to avoid physical exertion and must not perform exercise training. This study aims to evaluate the effectivity and safety of a low-dose training program in patients with pulmonary hypertension and rheumatic disease.

• Pulmonary Hypertension
• Connective Tissue Disease

• Active Comparator: exercise training group
  exercise and respiratory therapy with specific program for pulmonary hypertension (respiratory therapy, dumbbell training, ergometer training, mental training)
  Intervention: Behavioral: exercise training
• No Intervention: Control group without exercise training
  continuation of sedentary lifestyle without advice for specific exercise training

Inclusion Criteria:

1. Informed consent
2. Men and women 18 - 80 years
3. Diagnosed rheumatic disease: rheumatoid arthritis, Collagenosis (Systemic Lupus Erythematodes, Systemic Sclerosis, Sjögren-Syndrome, Sharp-Syndrome, Crest-Syndrome, Mixed connective tissue disease)

4. Symptomatic PAH (WHO- functional class II-IV) invasively diagnosed by right heart catheterisation

   • Mean pulmonary artery pressure (mPAP) > 25 mmHg
   • Pulmonary capillary wedge pressure (PCWP) > 15 mmHg
   • Pulmonary vascular resistance (PVR) at baseline >320 dyn.sec/cm5 patients under optimized medical treatment since at least 2 ½ months

Exclusion Criteria:

1. Other forms of PAH.
2. Pregnancy or lactation
3. Change in medication during the last 2 ½ months
4. Patients with signs of right heart decompensation
5. Severe impairment of walking
6. Unclear diagnosis
7. No invasive diagnosis of PH
8. Acute illness, infection, fever
9. Severe lung disease with FEV1 <50% and TLC< 70% below reference