Raynaud Phenomenon Resistant to the Initial Therapy in Patient with Systemic Lupus Erythematosus

Author : H. Susilo, R.M. Budiarto, H. Anugrawan, R. Amalia
Upload Date : 19-04-2018

Introduction: The Raynaud phenomenon is an exaggerated vascular response to cold temperatures or emotional stresses such as abnormal vasoconstriction of digital arteries and cutaneous arterioles. Sometimes the patient is resistant to initial therapy thus it requires surgical sympathetic blockade to prevent and treat digital ischemia.

Objective: The purpose of this case report is to present a patient with Raynaud phenomenon that is resistant to initial therapy thus requiring surgical sympathectomy.

Case Ilustration: A woman, 28 y.o., was referred to Dr. Soetomo Hospital with pain at the fingertips of her extremities especially when being exposed to cold. Since last month, her four right-hand fingertips had blackening. BP: 100/60 mmHg; HR: 124 bpm. Physical examination was anemic, splenomegaly, necrotic four right-hand fingertips. Echocardiography showed mild MR, LV dilatation (LVIDd 5,6cm), EF 30%, global LV hypokinetic, eccentric LVH, mild pericardial effusion. Vascular ultrasound was within normal limits. Laboratory test showed HB: 10.4; WBC: 2.600; PLT: 137.000; C3: <16; C4: <6,0; ANAtest: 270,11. The patient was given warming therapy, necrotic fingertip amputation, Diltiazem, Ramipril, Beraprost, and Pentoxifylline. Once being diagnosed as Systemic Lupus Erythematosus (SLE), the patient received high dose of Methylprednisolone and Cyclophosphamide. Because of still being symptomatic, thoracic sympatectomy was decided to be performed. After this procedure, the patient had reduced symptoms.

Discussion: The assessment of clinical response to initial therapy was performed by asking the patient to consider the impact of the conditions on her life, the frequency and duration of attacks, and the resulted disability. Resistance to initial therapies includes general nonpharmacologic measures, maximum tolerated doses of CCBs, and other vasodilator agents. In patients with critical digital ischemia, sympathectomy is recommended when medical therapy does not give good outcome.

Conclusion: We reported a patient with Raynaud phenomenon with SLE and dilated cardiomyopathy that was not improved with initial and causative therapy so a surgical thoracic sympathectomy was performed.

KEYWORDS : Raynaud phenomenon, systemic lupus erythematosus, resistant to initial therapy, thoracic sympathectomy

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