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Raynaud’s phenomenon

Raynaud’s phenomenon (RF) is a clinical presentation of vasospasm of the small arteries and arterioles of fingers and toes provoked by cold exposure and emotional stress. RP may affect also lips, earlobes, nose and other acral parts of the body. It manifests usually in three phases: ischemia, asphyxia and reactive hyperemia with skin discolouration from pallor to cyanosis and finally redness when blood flow is recovered. A sharp demarcation between the affected and unaffected areas is a characteristic feature. RP is primary and secondary. Secondary RP is observed in a number of rheumatic diseases e. g., systemic sclerosis (SSc), mixed connective tissue disease (MCTD), undifferentiated connective tissue disease (UCTD), dermatomyositis/polymyositis (DM/PM), systemic lupus erythematosus (SLE), Sjögren syndrome, systemic vasculitis, rheumatoid arthritis. RP is also a clinical symptom in various non-rheumatic conditions. Primary considerations in rheumatologic practice are drug-induced RP (beta blockers, cytotoxic drugsvinblastine, bleomycin, interferon) and paraneoplastic RP (in solid tumors and haematological malignancies).


            The diagnosis of primary RP can be established in these cases, in which no underlying causes for the development of a vasospastic disorder can be identifed. Women are 4–20 times more likely then men to develop primary RP. The onset of the disease is usually at puberty and is more frequent in certain families. Symmetrical vasospastic attacks and a benign course without trophic lesions is typical. Primary RP is often associated with migraine, retinopathy and Prinzmetal’s angina. The absence of an abnormal capillaroscopic pattern is one of the diagnostic criteria for primary RP. For the diagnosis of primary RP the lack of the following criteria (Le Roy and Medsger) is required (a) digital ulcerations and gangrenes (b) elevated erythrocyte sedimentation rate (ESR); (c) positive test for antinuclear antibodies (ANA) with a high titer (d) abnormal capillaroscopic pattern.


            Older age of onset – usually after 30 years, clinical features for autoimmune disease, trophic alterations of the fngers and toes, positive autoantibodies and abnormal capillaroscopic findings are the characteristic features of secondary RP in rheumatic diseases. Among rheumatic diseases, RP is one of the most frequent symptoms in SSc with the frequency of approximately 90–95% and is usually the initial symptom that precedes other features of the disease by years. RP in SSc is severe and often presents with digital ulcers.


The frequency of RP in MCTD is about 75-96% and in UCTD – over 80%. In UCTD patients, there are signs of systemic rheumatic diseases without fulfillment the criteria of a definite rheumatic disorder. The prevalence of RP in DM and PM is over 20%, but digital necroses are not observed and if this is present, underlying malignancy should be suspected. The prevalence of RP in SLE is reported to be ranging from 10 to 45% and it usually has a more benign course without tissue necrosis


            RP may also be a paraneoplastic symptom. A particular vigilance is required in new-onset symptoms after the age of 60. Ischemia of the fingers is observed in patients with solid tumors (carcinomas of breast, stomach and esophagus) as well as in oncohaematologic diseases (multiple myeloma, thrombocythemia, etc.).


            Laboratory and instrumental investigations

            The obligatory screening of RP patients for differentiation of primary RP from secondary RP in rheumatic diseases includes complete blood count, inflammatory markers, ANA test and nailfold capillaroscopy. Other investigations are ordered at the discretion of the physician in clinical context.


                The nailfold capillaroscopy is a key method for diagnosis and differentiation of primary and secondary RP in rheumatic diseases, which is of crucial importance because of the different severity, prognosis and the different therapeutic approach. The absence of an abnormal capillaroscopic pattern is one of the diagnostic criteria for primary RP. The findings are interpreted in clinical context. The investigation should be performed in all RP patients even when clinical and laboratory signs for connective tissue disease are absent, because the abnormal capillaroscopic pattern inherits a high positive predictive value for development of systemic rheumatic disease. RP patients should be monitored via nailfold capillaroscopy on a regular basis in order to detect those cases that will develop systemic rheumatic disease during the follow-up. The intervals of follow-up are over 6 months as appearance of structural capillary alterations usually takes a longer period of time. This recommendation is not valid in cases when new alarming symptoms occur, when the investigation is indicated to be performed earlier in clinical context.


            The treatment of RP depends on its clinical form and severity and includes various classes vasodilators (ginkgo biloba, calcium channel blockers, etc), other drug classes in physician’s judgement and proper hygienic regimen.


                                                                                              Dr Sevdalina Lambova, MD, PhD