CLINICALLY RELEVANT DIFFERENCES BETWEEN PRIMARY AND SECONDARY RAYNAUD'S PHENOMENON SECONDARY TO CONNECTIVE TISSUE DISEASES: RESULTS FROM A LARGE INTERNATIONAL SURVEY.

Di Donato, S.; Huang, S.; Pauling, J.; Sabbagh, M.; Del Galdo, F.; Khanna, D.; Hughes, M.

CLINICALLY RELEVANT DIFFERENCES BETWEEN PRIMARY AND SECONDARY RAYNAUD'S PHENOMENON SECONDARY TO CONNECTIVE TISSUE DISEASES: RESULTS FROM A LARGE INTERNATIONAL SURVEY.

All Authors

Di Donato, S.

Huang, S.

Pauling, J.

Sabbagh, M.

Del Galdo, F.

Khanna, D.

Hughes, M.

LTHT Author

Del Galdo, Francesco

LTHT Department

NIHR Leeds Biomedical Research Centre
Rheumatology

Publication Date

2024

Item Type

Conference Abstract

Abstract

Introduction: Raynaud's phenomenon (RP) is a symptom complex associated with digital vascular compromise classically characterized by colour change, and neurosensory symptoms. In the majority of individuals, RP is primary (PRP), or idiopathic, but can occur secondary (SRP) to a broad range of underlying conditions, including connective tissue disease (CTD). RP significantly impacts on quality of life and, in more severe cases, terminate in irreversible digital ischaemia. Our aim was to examine for clinically relevant differences between PRP and SRP (CTD-associated) concerning the patient experience including physical characteristics, ischaemic sequelae, and treatment. Material(s) and Method(s): We report cross-sectional results from the Patient Survey of experiences of Raynaud's Phenomenon (PASRAP), which aimed to explore the broad-ranging impact of RP. The survey was widely distributed online including via social medial. Participation was voluntary and responses were anonymous. Result(s): 1229 respondents completed PASRAP with selfreported RP: PRP 218 (17.7%) and SRP 1011 (82.3%) secondary to CTD. The majority (n= 747) of respondents had SSc: 260 dcSSc (34.8%) and 402 (53.8%) lcSSc, along with 85 (11.4%) SSc-overlap. Only SRP patients had experienced previous digital ulcers (36.9%) or gangrene (20.1%). The mean (SD) age was significantly lower in respondents with PRP (41.7 [11.8] vs 54.2 [12.4], p<0.0001). Only half of respondents felt that their symptoms were adequately controlled by their current medication(s), more commonly in SRP (45.2% vs 55.2%, p=0.0084). During attacks, more subjects with SRP reported cyanotic colour changes (92.2% vs 86.5%, p=0.0089). However, patients with PRP experienced more pain (72.1% vs 55.9%, p<0.0001), numbness (80.3% vs 69.4%, p=0.0016), stinging/throbbing (93.4% vs 80.8%, p<0.0001), and tingling (84.0% vs 77.5%, p=0.0345). A higher proportion of subjects with SRP presented with blue/purple colour changes of the fingers in response to cold (82.5% vs 64.8%, p<0.0001) and stress (67.2% vs 46.9%, p<0.0001). A higher proportion of patients with PRP reported RP attacks without the fingers changing colour (45.0% vs 25.6%, p<0.0001) and complete resolution of symptoms/ a restored sensation between RP attacks (69.9% vs 53.6%, p=0.0003). Most respondents (PRP and SRP) could identify a visible boundary between normal/ abnormal coloured skin with RP attacks (88.0% vs 83.2%, p=0.0849). Conclusion(s): Patients with CTD-SRP are older compared to PRP and present with more profound ischaemic colour change with cyanosis during RP attacks in response to cold and stress, reflecting the severe vasculopathy of SRP, and less complete resolution of symptoms between attacks. Patients with PRP have more neurosensory features.