Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content + Download Section PDF Listen ++ For further information, see CMDT Part 20-10: Raynaud Phenomenon + Key Features Download Section PDF Listen +++ +++ Essentials of Diagnosis ++ Paroxysmal bilateral digital pallor and cyanosis followed by rubor Precipitated by cold or emotional stress; relieved by warmth Primary form: benign course; usually affects young women Secondary form: can cause digital ulceration or gangrene +++ General Considerations ++ Raynaud phenomenon (RP) is a syndrome of paroxysmal digital ischemia Most commonly caused by an exaggerated response of digital arterioles to cold or emotional stress Toes and other acral areas (eg, nose and ears) can be affected as well as fingers Classified as primary (idiopathic or Raynaud disease) or secondary Primary form Appears between ages 15 and 30, almost always in women Tends to be mildly progressive +++ Demographics ++ Primary RP occurs in 2–6% of adults + Clinical Findings Download Section PDF Listen +++ +++ Symptoms and Signs ++ Only one or two fingertips may be affected in early attacks All fingers down to the distal palm may be involved as condition progresses Thumbs are rarely affected Well-demarcated digital pallor or cyanosis in initial phase Intense rubor, throbbing, paresthesia, pain, and slight swelling during recovery phase Patient usually asymptomatic between attacks Sensory changes that often accompany vasomotor manifestations Numbness Tingling Diminished sensation Aching pain Primary RP Symmetric involvement of the fingers of both hands Spasm becomes more frequent and prolonged Does not cause digital pitting, ulceration, or gangrene Secondary RP May be unilateral and may involve only one or two fingers Nailfold capillary abnormalities Digital pitting or ulceration Skin tightening Loss of extremity pulse Rash Swollen joints +++ Differential Diagnosis ++ RP occasionally the first manifestation of such rheumatic conditions as Systemic sclerosis (including its CREST variant) Systemic lupus erythematosus Mixed connective tissue disease Thromboangiitis obliterans (Buerger disease) Thoracic outlet syndromes Acrocyanosis Frostbite Ergot poisoning (unusual) Bleomycin and vincristine chemotherapy Cryoglobulinemia Erythromelalgia can mimic the rubor phase of RP; exacerbation by heat and relief with cold readily distinguish erythromelalgia from RP + Diagnosis Download Section PDF Listen +++ +++ Laboratory Tests ++ Serologic tests for rheumatic diseases (Table 20–7) ++Table Graphic Jump LocationTable 20–7.Frequency (%) of autoantibodies in rheumatic diseases.1View Table||Download (.pdf) Table 20–7. Frequency (%) of autoantibodies in rheumatic diseases.1 ANA Anti-Native DNA Rheumatoid Factor Anti-Sm Anti-SS-A Anti-SS-B Anti-SCL-70 Anti-Centromere Anti-Jo-1 ANCA Rheumatoid arthritis 30–60 0–5 70 0 0–5 0–2 0 0 0 0 Systemic lupus erythematosus 95–100 60 20 10–25 15–20 5–20 0 0 0 0–1 Sjögren syndrome 95 0 75 0 65 65 0 0 0 0 Diffuse scleroderma > 95 0 30 0 0 0 33 1 0 0 Limited scleroderma (CREST syndrome) > 95 0 30 0 0 0 20 50 0 0 Polymyositis/dermatomyositis 80 0 33 0 0 0 0 0 20–30 0 Granulomatosis with polyangiitis 0–15 0 50 0 0 0 0 0 0 93–961 1Frequency for generalized, active disease.ANA, antinuclear antibodies; Anti-Sm, anti-Smith antibody; anti-SCL-70, anti-scleroderma antibody; ANCA, antineutrophil cytoplasmic antibody; CREST, calcinosis cutis, Raynaud phenomenon, esophageal motility disorder, sclerodactyly, and telangiectasia. +++ Diagnostic Procedures ++ Evaluate nailfold capillary pattern Place a drop of grade B immersion oil at cuticle View area with an ophthalmoscope set to 20–40 diopters Dropout of capillaries and dilation of the remaining capillary loops indicate the patient has a secondary form of RP, most commonly scleroderma While highly specific for secondary RP, nailfold capillary changes have a low sensitivity + Treatment Download Section PDF Listen +++ +++ Medications ++ Calcium channel blockers First-line therapy Produce a modest benefit More effective in primary RP than secondary RP Slow release nifedipine (30–180 mg/day orally) or amlodipine (5–20 mg/day orally), felodipine, isradipine, or nisoldipine are popular and more effective than verapamil, nicardipine, and diltiazem Other medications that are sometimes effective Angiotensin II receptor blockers Topical nitrates Phosphodiesterase inhibitors (eg, sildenafil, tadalafil, and vardenafil) Selective serotonin reuptake inhibitors (fluoxetine) Statins Endothelin-receptor inhibitors (ie, bosentan) Severe or refractory episodes can be treated with intravenous infusions of prostacyclin or prostacyclin analogs, such as Epoprostenol Iloprost Treprostinil +++ Surgery ++ Sympathectomy indications Frequent and severe attacks Interference with work and well being Development of trophic changes and failure of medical measures Cervical sympathectomy is modestly effective for primary but not secondary RP Digital sympathectomy may improve secondary RP +++ Therapeutic Procedures ++ Keep the body warm Wear gloves or mittens in temperatures that precipitate attacks Protect hands from injury at all times Softening and lubricating lotion Smoking cessation Avoidance of sympathomimetic drugs (eg, decongestants, diet pills, and amphetamines) + Outcome Download Section PDF Listen +++ +++ Prognosis ++ Primary RP is benign and largely a nuisance for affected individuals Prognosis of secondary RP depends on severity of the underlying disease +++ Prevention ++ Avoidance of cold exposure Reduction of emotional stress +++ When to Refer ++ Appropriate management of patients with secondary RP often requires consultation with a rheumatologist +++ When to Admit ++ Patients with severe digital ischemia as evidenced by demarcation should be admitted for intensive therapy + References Download Section PDF Listen +++ + +Bello RJ et al. The therapeutic efficacy of botulinum toxin in treating scleroderma-associated Raynaud's phenomenon: a randomized, double-blind, placebo-controlled clinical trial. Arthritis Rheumatol. 2017 Aug;69(8):1661–9. [PubMed: 28426903] + +Pauling JD et al. Raynaud's phenomenon—an update on diagnosis, classification and management. Clin Rheumatol 2019 Dec;38(12):3317–30. [PubMed: 31420815] + +Roustit M et al. On-demand sildenafil as a treatment for Raynaud Phenomenon: a series of n-of-1 trials. Ann Intern Med. 2018 Nov 20;169(10):694–703. [PubMed: 30383134]