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Vascular acrosyndromes in young adult population. Definition of clinical symptoms and connections to joint hypermobility

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Abstract

Objectives

Clinical recognition of vascular acrosyndromes is often challenging. The term Raynaud’s phenomenon (RP) is commonly overused to describe any form of cold-related disorder. This study aims to formally evaluate peripheral vascular symptoms affecting the population, aged ≤ 40 years, and identify any correlations to joint hypermobility (JH).

Patients and methods

Fifty patients (31 males, 19 females) with vasomotor symptoms enrolled in this five-year prospective observational study. Clinical examination by a rheumatologist and a vascular surgeon was performed along with cardiology, echocardiographic and Doppler evaluation. Patients underwent blood cell count, biochemistry, thyroid and selectively immunologic testing. Twenty-four (48%) of them performed nailfold capillaroscopy. The SPSS for Windows, v.17.0, Chicago, USA, was used for the statistical analyses.

Results

Twenty-eight patients (56%) presented with erythromelalgia (EM), 6 (12%) with acrocyanosis (AC) and 9 (18%) as a combination of the above disorder. RP diagnosed in five (10%) while two patients (4%) presented as a mix of EM-RP. There was no correlation with abnormal laboratory tests. Increased incidence of JH was found in EM and AC patients. Among those who were tested with nailfold capillaroscopy, 75% had abnormalities ranged from mild to autoimmune-like diseases.

Conclusions

Erythromelalgia is the commonest functional vasculopathy in young population followed by acrocyanosis and a combination of these conditions. Joint hypermobility is markedly increased, indicating that dysautonomy may be considered the causative factor following a trigger event. Overall, RP was observed in 14% of patients. Clinical recognition of these disorders avoids unnecessary investigation.

Key Points

• Vascular acrosyndromes in young adults are commonly functional disorders resembling vascular algodystrophy induced by thermic stress.

• Dysautonomy of joint hypermobility is the co-factor influencing the appearance of the vascular disorders.

• Raynaud’s phenomenon accounts to approximately 14% of vascular acrosyndromes presented in the young adult population.

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Funding

The study was supported by the Hellenic Rheumatology Society.

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Authors and Affiliations

  1. Rheumatology Department, 424 General Military Hospital, Nea Efkarpia, 56249, Thessaloniki, Greece

    Periklis Vounotrypidis, Grigorios T. Sakellariou, Dimitrios Zisopoulos, Nikoleta Kefala & Charalambos Bermperidis

  2. 2nd Propaedeutic Clinic, Department of Internal Medicine, “Ippokration” Hospital, Thessaloniki, Greece

    Athina Pyrpasopoulou & Spyros Aslanidis

  3. Department of Immunology and Microbiology, 424 General Military Hospital, Thessaloniki, Greece

    Dimitrios I. Oikonomou

  4. Cardiology Department, 424 General Military Hospital, Thessaloniki, Greece

    Constantinos Stefanis

  5. Vascular Surgery Department, 424 General Military Hospital, Thessaloniki, Greece

    Periklis Pappas

Authors
  1. Periklis Vounotrypidis
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  2. Athina Pyrpasopoulou
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Correspondence to Periklis Vounotrypidis.

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Patients were asked to participate in this prospective observational study and written informed consent was obtained, according to the Declaration of Helsinki. The study was approved by the Hospital’s Ethical Committee.

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Vounotrypidis, P., Pyrpasopoulou, A., Sakellariou, G.T. et al. Vascular acrosyndromes in young adult population. Definition of clinical symptoms and connections to joint hypermobility. Clin Rheumatol 38, 2925–2932 (2019). https://doi.org/10.1007/s10067-019-04627-w

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  • DOI: https://doi.org/10.1007/s10067-019-04627-w

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