Abstract Ref Number = APCP741
A Case Series of Pediatric Secondary Raynaud’s Phenomenon in Cipto Mangunkusumo Hospital
Fatimah Saidah ,Nia Kurniati,Dina Muktiarti
RSCM Department of Child Health, Faculty of Medicine, Universitas Indonesia, Cipto Mangunkusumo National General Hospital, Jakarta, Indonesia
Background : Raynaud’s phenomenon (RP) is an exaggerated vasospasm in response to cold temperature or emotional stress. It is considered as primary RP when there is no evidence of any associated disorder or secondary in the presence of underlying diseases. This case series reports seven cases of secondary Raynaud’s phenomenon that illustrates the variation of the underlying diseases and a brief review of their clinical presentation, diagnosis and therapeutic approach.
Case Presentation Summary : Seven cases of RP secondary to systemic lupus erythematosus (SLE), polyarteritis nodosa (PAN), juvenile dermatomyositis (JDM), antiphospholipid syndrome (APS), and streptococcal infection were reported. The clinical manifestations of RP varied from reversible painful cyanotic digits to severe digital necrosis in four patients with SLE, PAN, and streptococcal infection. Laboratory work up was determined by suspected underlying diseases based on sign and symptoms, with anti-nuclear antibody (ANA) being the most regularly tested marker to detect secondary connective tissue disease and anti-streptolysin O (ASTO) to rule out streptococcal-related vasculitis. ANA was positive in four cases while three cases in our series showed positive ASTO. Four patients with necrotic digits underwent Doppler ultrasonography. All patients received steroid and other treatment for the underlying diseases. Patient with mild manifestations of RP responded well with calcium channel blocker alone or in conjunction with phosphodiesterase inhibitor, while more severe manifestations such as necrotic digits needed intravenous heparin followed by oral warfarin to prevent further development of gangrene.
Learning Points/Discussion : Sign of vasospasm should raise a suspicion of RP. All of the RP cases in our setting were secondary to various underlying conditions. Other clinical manifestations and specific laboratory findings such as ANA and ASTO were required to distinguish one underlying condition to another. Aggressive management of RP correlates with the disease prognosis.
Keywords: Raynaud’s phenomenon vasculitis children