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Raynaud's phenomenon-an update on diagnosis, classification and management
John D Pauling1,2, Michael Hughes3, Janet E Pope4
1Royal National Hospital for Rheumatic Diseases (at Royal United Hospitals), Upper Borough Walls, Bath, BA1 1RL, UK. JohnPauling@nhs.net.
Clinical Rheumatology
|August 18, 2019
Summary
Raynaud's phenomenon involves digital vascular issues, with primary and secondary causes. Understanding its varied origins is key for effective management and research into this condition.
Area of Science:
- Vascular Medicine
- Rheumatology
- Clinical Diagnosis
Background:
- Raynaud's phenomenon (RP) is a clinical diagnosis of digital vascular compromise, often relying on patient self-report due to its episodic nature.
- Primary RP lacks demonstrable underlying pathology, potentially encompassing functional vasospasm, thermoregulatory responses, or subclinical atherosclerosis.
- Secondary RP is linked to diverse pathologies, including rheumatological, hematological, endocrinological, and vascular conditions, with systemic sclerosis (SSc) being a significant focus.
Purpose of the Study:
- To review the causes, pathogenesis, clinical features, classification, and management of Raynaud's phenomenon.
- To outline a practical approach for evaluating and managing RP.
- To identify knowledge gaps and unmet research needs in RP.
Main Methods:
- Literature review examining causes, pathogenesis, clinical features, classification, and management of RP.
- Analysis of current understanding of primary and secondary RP.
- Discussion of the patient experience and its implications for clinical trials.
Main Results:
- Raynaud's phenomenon is a symptom complex with diverse etiopathogenic processes, not a single entity.
- Systemic sclerosis-associated RP involves vasospasm compounded by obliterative microangiopathy, leading to severe digital ischemia.
- The episodic paradigm of RP is being challenged, impacting patient-reported outcome measures in clinical trials.
Conclusions:
- Effective management of Raynaud's phenomenon requires understanding its varied causes and underlying pathologies.
- Further research is needed to address knowledge gaps and refine the classification and treatment of RP.
- Raynaud's phenomenon significantly contributes to morbidity, particularly in autoimmune rheumatic diseases like systemic sclerosis.