Investigation of Coronary and Pulmonary Circulatory Parameters in Systemic Sclerosis
Condition: Systemic Sclerosis
MeSH terms: Scleroderma, Systemic; Scleroderma, Diffuse; Sclerosis
Study type: Observational

Summary: 
      Aim: Determination of frequency and nature of coronary involvement (including epicardial and
      microvascular) among scleroderma patients referred for right heart catheterization.
    

Detailed description: 
      Systemic sclerosis (SSc)is a multiorgan autoimmune disease characterized by fibrosis of the
      skin and internal organs. Cardio-pulmonary manifestation in systemic sclerosis (SSc) patients
      has significant impact on the prognosis. Several elements of the heart and lung involvement
      encountered frequently in combination. Clinically apparent SSc cardiac involvement is
      recognized as poor prognostic factor, and in our experience it is the most frequent cause of
      death among SSc patients. Moreover arrhythmias, diastolic and less frequently systolic
      dysfunction considerably interfere with the quality of life of these patients. Cardiac
      involvement includes primary cardiac disorders like fibrosis of the contractile elements,
      ischemia due to the involved microcirculation and/or to the epicardial coronaries and some
      secondary ones like consequences of systemic or pulmonary hypertension.

      Autopsy identifies fibrosis of the myocardium in up to 80 percent of cases. Its distribution
      is unrelated to epicardial coronary stenosis, and inflammatory processes are thought to play
      substantial role in its evolution. Microvascular disease (MVD) that causes reduction of the
      vasodilatory capacity independently from epicardial coronary disease (CAD), is known for
      several decades. Short term benefits from vasodilatory treatment has been also reported in
      some latest studies.

      Symptoms of the elements of cardio-pulmonary involvement overlap and coronary abnormalities
      alike MVD were also found in asymptomatic patients. Recently prevalence of CAD is reported to
      be similar to gender, age and symptom matched controls. Atypical clinical presentations were
      predominant and nevertheless these findings indicate that SSc patients are not free from CAD.

      Clarification the causes of reduced exercise capacity, chest pain and faint in SSc is a
      challenging task and requires multidisciplinary approach. Driven by its high mortality and
      due to the recent emergence of effective therapy SSc patients are regularly screened for
      pulmonary hypertension (PAH). Clinical presentation of scleroderma related PAH is unspecific.
      Fatigue, chest pain and dyspnea may be related to other organs, such as the lung,
      musculoskeletal system or the esophagus. The definitive diagnosis of PAH still depends on the
      results of right heart catheterization (RHC). As the non-invasive diagnostics are influenced
      and to some extent restricted by multiple clinical manifestations, RHC is performed liberally
      in symptomatic patients. Still a considerable proportion of patients who underwent RHC have
      normal pulmonary pressure and the cause of symptoms may remain obscure as cardiac involvement
      may not be correctly diagnosed or entirely overlooked.

      We intend to characterize coronary artery disease in symptomatic SSc patients by means of a
      prospective cross-sectional study consisting of organized screening of SSc patients and
      systematic left heart catheterization (coronary angiography, and coronary flow reserve
      estimation) in all patients referred to RHC. Prognostic impact of the different
      manifestations are determined during five-year follow-up of the study.
    

Observational model: Case-Crossover
Time perspective: Prospective
Sampling method: Probability Sample
Study population: 
        Systemic sclerosis
      
Biospecimen description: 
      Serum samples
    
Keywords: atherosclerosis; pulmonary hypertension