Literature DB >> 31177398

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

Periklis Vounotrypidis1, Athina Pyrpasopoulou2, Grigorios T Sakellariou3, Dimitrios Zisopoulos3, Nikoleta Kefala3, Dimitrios I Oikonomou4, Constantinos Stefanis5, Spyros Aslanidis2, Charalambos Bermperidis3, Periklis Pappas6.   

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.

Entities:  

Keywords:  Acrocyanosis; Capillaroscopy; Dysautonomy; Erythromelalgia; Raynaud’s phenomenon

Mesh:

Year:  2019        PMID: 31177398     DOI: 10.1007/s10067-019-04627-w

Source DB:  PubMed          Journal:  Clin Rheumatol        ISSN: 0770-3198            Impact factor:   2.980


  32 in total

1.  Evolving Symptom Characteristics of Raynaud's Phenomenon in Systemic Sclerosis and Their Association With Physician and Patient-Reported Assessments of Disease Severity.

Authors:  John D Pauling; Elizabeth Reilly; Theresa Smith; Tracy M Frech
Journal:  Arthritis Care Res (Hoboken)       Date:  2019-07-03       Impact factor: 4.794

Review 2.  Raynaud's syndrome in children: systematic review and development of recommendations for assessment and monitoring.

Authors:  Clare E Pain; Tamás Constantin; Natasa Toplak; Monica Moll; Christof Iking-Konert; Daniella P Piotto; Nuray Aktay Ayaz; Dana Nemcova; Peter H Hoeger; Maurizio Cutolo; Vanessa Smith; Ivan Foeldvari
Journal:  Clin Exp Rheumatol       Date:  2016-07-27       Impact factor: 4.473

3.  Validity and reliability of three methods used in the diagnosis of Raynaud's phenomenon. The UK Scleroderma Study Group.

Authors:  P Brennan; A Silman; C Black; R Bernstein; J Coppock; P Maddison; T Sheeran; C Stevens; F Wollheim
Journal:  Br J Rheumatol       Date:  1993-05

Review 4.  Acrocyanosis: the Flying Dutchman.

Authors:  Andrew K Kurklinsky; Virginia M Miller; Thom W Rooke
Journal:  Vasc Med       Date:  2011-03-22       Impact factor: 3.239

5.  A systematic review of vitamin D status in southern European countries.

Authors:  Yannis Manios; George Moschonis; Christina-Paulina Lambrinou; Konstantina Tsoutsoulopoulou; Panagiota Binou; Alexandra Karachaliou; Christina Breidenassel; Marcela Gonzalez-Gross; Mairead Kiely; Kevin D Cashman
Journal:  Eur J Nutr       Date:  2017-10-31       Impact factor: 5.614

Review 6.  Raynaud syndrome.

Authors:  Sergio Toledo Valdovinos; Gregory J Landry
Journal:  Tech Vasc Interv Radiol       Date:  2014-11-13

7.  Different factors influencing the expression of Raynaud's phenomenon in men and women.

Authors:  L Fraenkel; Y Zhang; C E Chaisson; H R Maricq; S R Evans; F Brand; P W Wilson; D T Felson
Journal:  Arthritis Rheum       Date:  1999-02

8.  Dysautonomia in the joint hypermobility syndrome.

Authors:  Yael Gazit; A Menahem Nahir; Rodney Grahame; Giris Jacob
Journal:  Am J Med       Date:  2003-07       Impact factor: 4.965

Review 9.  Raynaud's phenomenon: a proposal for classification.

Authors:  E C LeRoy; T A Medsger
Journal:  Clin Exp Rheumatol       Date:  1992 Sep-Oct       Impact factor: 4.473

10.  Autoantibodies and microvascular damage are independent predictive factors for the progression of Raynaud's phenomenon to systemic sclerosis: a twenty-year prospective study of 586 patients, with validation of proposed criteria for early systemic sclerosis.

Authors:  Martial Koenig; France Joyal; Marvin J Fritzler; André Roussin; Michal Abrahamowicz; Gilles Boire; Jean-Richard Goulet; Eric Rich; Tamara Grodzicky; Yves Raymond; Jean-Luc Senécal
Journal:  Arthritis Rheum       Date:  2008-12
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  1 in total

1.  Primary Erythromelalgia Complicated by Cellulitis: A Case Report and Review of Literature.

Authors:  Sara Sharif; Lubaina Haider; Latoya Freeman; Isabel M McFarlane
Journal:  Am J Med Case Rep       Date:  2020-04-05
  1 in total

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