Abstract
Raynaud’s phenomenon (RP) is a common clinical syndrome in rheumatology and represents a clinical sequel of recurrent reversible vasospasm of small peripheral arteries and arterioles provoked by cold exposure and emotional stress. RP is classified into two major categories – primary RP in the absence of an underlying disease and secondary RP that is in the context of another disease. The differentiation between primary and secondary RP is of crucial importance in clinical practice because patients with primary RP are with benign course while those with secondary RP require further differentiation and establishment of the precise diagnosis and treatment. The diagnosis of RP is clinical and some clinical features (severity of vasospastic attacks, thumb involvement, appearance of digital ulcers or necrosis) differentiate primary from secondary RP. However, precise differential diagnosis between primary and secondary RP requires laboratory assessment including immunologic screening and capillaroscopic examination. In primary RP, the pathological microcirculation with inadequate acclimatization prior to capillaroscopic examination may lead to visualization of comma-like appearance of capillaries, interrupted blood flow and their morphology could not be adequately analyzed. Moreover, the zones with compromised perfusion may be wrongly interpreted as avascular areas. The skin temperature should be normal during capillaroscopic examination to avoid mistakes related to intermittent changes in the blood flow. Our experience and current literature data suggest that the diagnosis primary RP is confirmed in overall context in the absence of capillaroscopic features of microangiopathy. However, minor microvascular changes are common findings especially presence of dilated capillaries that might be related to altered vascular tone as a consequence of recurrent vasospastic attacks. In rare cases “scleroderma” type microangiopathy (i.e., giant capillaries, avascular areas, neoangiogenesis in association with devascularization and capillary derangement) could be observed in patients in whose clinical symptoms, laboratory and immunological findings are compatible with the diagnosis primary RP. They should be labeled “suspected secondary RP” and require close follow-up.
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