Pulmonary Arterial Hypertension in Systemic Sclerosis: Swift Diagnosis and Effective Treatment

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Pulmonary hypertension is becoming one of the main causes of death in patients with SSc, in sync with severe pulmonary fibrosis and surpassing scleroderma renal crisis.
Pulmonary hypertension is becoming one of the main causes of death in patients with SSc, in sync with severe pulmonary fibrosis and surpassing scleroderma renal crisis.

Risk factors for LHD include age ≥60 years, presence of valvular heart disease, reduced left ventricular ejection fraction (LVEF), evidence of elevated LV pressures (LV hypertrophy [LVH], diastolic dysfunction, left atrial enlargement ([LAE]), and comorbidities supporting LV disease such as diabetes, hypertensive heart disease, coronary artery disease, or obesity. Having no risk factors indicates a higher chance of PAH; the presence of 1 to 2 risk factors indicates likely PH-LHD; and having 3 or more risk factors indicates a higher chance of PH-LHD.

SSc-PAH accounts for approximately 15% to 30% of the total PAH population. Conditions to watch for in patients with rheumatic disease that may not be as common in the general population include:

  • Pulmonary veno-occlusive disease and/or pulmonary capillary hemangiomatosis
  • LV diastolic dysfunction
  • Chronic thromboembolic pulmonary hypertension (CTEPH)
  • Interstitial lung disease

Who to Test for SSc-PAH? Early Detection Is Crucial.

Clinical risk factors associated with SSc-PAH include:

  • Long-standing disease
  • Older age at scleroderma onset
  • Severe Raynaud phenomenon
  • Severe digital ischemia
  • Isolated low diffusing capacity of the lung for carbon monoxide (DLCO) <50%
  • FVC%/DLCO% >1.6
  • Increasing right ventricular systolic pressure (RVSP) >2 mm Hg per year
  • Anticentromere antibodies
  • Anti-U1 RNP antibodies
  • Anti-endothelial cell antibodies
  • Absence of anti-Sci-70 antibodies

Early detection of SSc-PAH is crucial, but the strength of their recommendation for these risk factors vary.

Researchers from the DETECT study created an algorithm to aid in the identification of patients best suited to assess for PAH with right heart catheterization.2 The goal of the research was to develop an evidence-based screening algorithm for PAH in patients with SSc that would minimize the number of missed PAH diagnoses, optimize the use of screening modalities, and optimize the use of diagnostic right heart catheterization.

The researchers assessed 500 patients from 18 countries to develop inclusion criteria associated with a total risk point score between 220 and 440. A score between 300 and 400 warranted a referral for echocardiogram; this diagnostic modality was not advised for patients with scores less than 300.

Another set of criteria were then applied to those who underwent echocardiogram to determine the necessity of right heart catheterization. The total risk point score ranged from 10 to 130. Patients with a score between 10 and 35 received no referral; those with a score between 35 and 135 received a referral for catheterization.

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