Prevalence and particularities of Raynaud’s phenomenon in patients with autoimmune desease

Abstract

Introduction: Raynaud's phenomenon (RP) is an episodic reversible peripheral ischemia usually provoked by cold or emotion.The phenomenon is named for Maurice Raynaud, who, as a medical student, defined the first case in 1862 as "episodic, symmetric, acral vasospasm characterized by pallor, cyanosis, suffusion, and a sense of fullness or tautness, which may be painful’’ Secondary RP should be distinguished from primary RP (Raynaud disease). Raynaud disease is characterized by the occurrence of the vasospasm alone, with no association with another illness. Secondary RP is a designation usually used in the context of vasospasm Associated with another illness, most commonly an autoimmune disease.Physical examination, nailfold capillaroscopy and immunological tests can differentiate primary from secondary RP. The prevalence of RP in most studies of the general population is between 3 and 5%. Primary RP is reversible vasospasm in peripheral arteries occurring in the absence of an underlying disease and accounts for 80–90% of cases.The prevalence of secondary RP is related to the underlying disease. Progression to secondary RP occurs in 14–37% of subjects with primary RP. Almost 99% of patients who progress develop an autoimmune disease. The aim of this study was to evaluate the prevalence and particularities of RP in patients with autoimmune disease. Materials and methods: All relevant information was obtained from the literature review. Discussion: RP is common in the general population, but may also be a sign of connective tissue disease. RP occurs in 98% of patients with systemic sclerosis (SSc) and may be their most pressing clinical problem; it occurs in 85% of patients with mixed CTD, between 10% and 45% of those with systemic lupus erythematosus, 33% of those with Sjögren syndrome, and 20% of those with polymyositis,dermatomyositis. In individuals with rheumatoid arthritis the overall prevalence is similar to that in the general population (10%); however, symptoms tend to be more severe. Conclusion: Prognosis of secondary RP is related to the underlying disease. Prognosis for the involved digit in these patients is related to the severity of the ischemia and the effectiveness of maneuvers to restore blood flow. Therefore it is important to look carefully for any underlying cause. More importantly, early intervention could improve the prognosis, such that, digital amputation caused of ischemic complications usually is not necessary if aggressive oral vasodilator therapy is initiated in patients with frequent or severe episodes RP.