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[Pulmonary hypertension in diffuse scleroderma and the CREST syndrome]
Plucne Bolesti : Casopis Udruzenja Pneumoftiziologa Jugoslavije = the Journal of Yugoslav Association of Phthisiology and Pneumology
|January 1, 1989
Summary
Pulmonary hypertension (PH) is more common in patients with CREST syndrome than diffuse systemic sclerosis. This study investigated PH prevalence in 22 patients, finding higher rates in CREST syndrome cases.
Area of Science:
- Cardiology
- Rheumatology
- Pulmonology
Context:
- Systemic sclerosis (SS) is a multisystem autoimmune disease.
- Pulmonary hypertension (PH) is a serious complication of SS.
- CREST syndrome and diffuse scleroderma are subtypes of SS with varying PH risks.
Purpose:
- To investigate the prevalence of manifest and latent pulmonary hypertension (PH) in patients with systemic sclerosis (SS).
- To compare the occurrence of PH in patients with diffuse scleroderma versus CREST syndrome.
- To determine the frequency of isolated PH in SS subtypes.
Summary:
- Microcatheterization revealed manifest PH in 19% of diffuse scleroderma and 50% of CREST syndrome patients.
- Latent PH was detected in 50% of diffuse scleroderma and 33% of CREST syndrome patients.
- PH occurred more frequently in CREST syndrome than diffuse scleroderma, consistent with literature. Isolated PH was more common in CREST syndrome.
Impact:
- Highlights the increased risk of pulmonary hypertension in CREST syndrome patients.
- Informs clinical suspicion and diagnostic strategies for PH in systemic sclerosis subtypes.
- Contributes to understanding the pathophysiology and clinical spectrum of SS-associated cardiopulmonary complications.