Raynaud phenomenon
Raynaud phenomenon is episodic digital vasospasm with triphasic pallor, cyanosis, and reactive hyperemia, split into a benign primary form and a secondary form driven by an underlying connective tissue disease. Diagnosis is clinical, with nailfold capillaroscopy and an autoantibody panel screening for systemic sclerosis, and treatment starts with cold avoidance before a dihydropyridine calcium channel blocker such as nifedipine.
- Topic reviewThe full article, organized the way the exam asks it.
- ABIM-format MCQsVignettes with an explanation for every wrong answer.
- Key factsThe numbers and thresholds worth memorizing, up front.
- AI tutorMedBrain answers the question you're stuck on.
What this article covers
- Overview
Definition: Raynaud phenomenon is reversible, episodic digital vasospasm producing a triphasic color change, pallor from ischemia, cyanosis from stasis, then rubor from reactive hyperemia, triggered by cold or emotional stress.
- Epidemiology
Primary disease: predominantly affects women, with onset typically before age 40 and symptoms stable over years.
- Risk factors
Female sex: dominant risk factor for primary disease.
- Pathophysiology
Exaggerated normal reflex: cold or emotional stress activates digital arteriolar vasoconstriction, a physiologic response that is exaggerated in Raynaud phenomenon.
- Clinical features
Attack pattern: discrete episodes of well-demarcated color change in one or more digits, precipitated by cold or stress and lasting minutes.
- Differential diagnosis
Systemic sclerosis: sclerodactyly, pitting scars, or an abnormal scleroderma-pattern capillaroscopy; absent Raynaud phenomenon with skin thickening makes this unlikely (systemic sclerosis).
- Diagnosis
Primary Raynaud phenomenon is a clinical diagnosis, established by cold- or stress-triggered triphasic color change with normal pulses, no ulcers or pitting, and no feature of an underlying disease; no laboratory test is required.
- Management
First, start non-pharmacologic measures: dress in layers, keep the whole body warm, wear insulated gloves, avoid abrupt cold exposure, stop smoking, and remove precipitating drugs such as beta-blockers and ergotamine.
- Complications
Digital ulceration: occurs in about 15% of patients with systemic sclerosis, typically on the fingertips, and heals slowly because of the underlying fixed vasculopathy.
- Prognosis
Primary disease is benign and stable: symptoms persist over years without progression to tissue loss, and most patients respond well to conservative measures and vasodilator therapy.
- Prevention
Maintain year-round core and extremity warmth with layered clothing, gloves, and socks, since core body cooling, not just hand cooling, provokes attacks.
Read the whole article
Subscribing to Internal Medicine opens every article, the full question bank and MedBrain, on the web, iPhone and Android.
Related articles
- Systemic sclerosis
Systemic sclerosis is a multiorgan disease of vascular injury and progressive fibrosis in which diffuse cutaneous disease extends skin thickening proximal to the elbows and knees and drives early visceral fibrosis. Diagnosis rests on the 2013 ACR/EULAR classification criteria and an antibody split (anti-Scl-70, anticentromere, anti-RNA polymerase III), and treatment is organ-specific: mycophenolate or nintedanib for interstitial lung disease, calcium channel blockers for Raynaud phenomenon, and captopril for scleroderma renal crisis.
- CREST syndrome
CREST syndrome (limited cutaneous systemic sclerosis) causes calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia, with skin thickening confined distal to the elbows and knees plus the face. Anticentromere antibody is the serologic hallmark, and annual echocardiographic screening for pulmonary arterial hypertension, treated with an endothelin receptor antagonist or phosphodiesterase-5 inhibitor, drives outcomes more than the renal or lung fibrosis risk of diffuse disease.
- Mixed connective tissue disease
Mixed connective tissue disease is an overlap syndrome defined by a high-titer anti-U1-ribonucleoprotein antibody together with clinical features of systemic lupus erythematosus, systemic sclerosis, and inflammatory myositis, with Raynaud phenomenon and puffy fingers as near-universal early findings. Treatment is organ-directed, built on hydroxychloroquine and glucocorticoids with methotrexate, azathioprine, or mycophenolate mofetil escalation guided by the dominant manifestation.
- Systemic lupus erythematosus
Systemic lupus erythematosus is a chronic multisystem autoimmune disease in which antinuclear and other autoantibodies drive immune-complex deposition and organ injury across skin, joints, kidneys, blood, serosa, and the nervous system. Diagnosis rests on a positive antinuclear antibody plus weighted 2019 EULAR/ACR criteria totaling 10 or more points. Hydroxychloroquine is lifelong first-line therapy for nearly every patient, with glucocorticoids and steroid-sparing immunosuppression added for moderate to severe or organ-threatening disease.
- Sjogren syndrome
Sjogren syndrome is a systemic autoimmune exocrinopathy in which lymphocytic infiltration of the lacrimal and salivary glands causes keratoconjunctivitis sicca and xerostomia, occurring alone (primary) or alongside another rheumatologic disease such as rheumatoid arthritis or lupus (secondary). Diagnosis combines the anti-Ro (SSA) and anti-La (SSB) antibody panel, objective dryness testing, and labial salivary gland biopsy under the 2016 ACR/EULAR classification criteria. Management centers on sicca-directed therapy, pilocarpine or cevimeline for xerostomia and topical cyclosporine for keratoconjunctivitis, with hydroxychloroquine or immunosuppression for extraglandular disease.