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This is a clinician-written, evidence-based summary aligned to the USMLE Step 2 CK Content Outline. It is intended for medical students preparing for USMLE Step 2 CK. Management reflects current ACC/AHA, USPSTF, and APA guidelines. Always cross-reference with UpToDate, institutional protocols, and clinical judgment.
The Bottom Line
- Systemic sclerosis = vasculopathy plus fibrosis
- Limited disease: anti-centromere and late PAH
- Diffuse disease: anti-Scl-70/RNA polymerase III, ILD, renal crisis risk
- Renal crisis = abrupt severe HTN and AKI; treat with ACE inhibitor
- High-dose steroids increase renal crisis risk
Overview
Systemic sclerosis is autoimmune microvascular injury and fibrosis affecting skin, GI tract, lungs, kidneys, and heart. Raynaud phenomenon is often the first feature.
Epidemiology
Uncommon, female predominant, typical onset age 30-60. Mortality is driven by ILD, PAH, and renal crisis.
Clinical Features
Symptoms
Raynaud phenomenon triggered by cold or stress
Skin tightening, puffy fingers, reduced hand function
GERD, dysphagia, bloating, constipation, or diarrhea
Dyspnea or dry cough from ILD or PAH
Abrupt severe hypertension, oliguria, or AKI suggesting renal crisis
Signs
Sclerodactyly and reduced oral aperture
Telangiectasias and calcinosis cutis
Digital pitting scars or ulcers
Tendon friction rubs
Bibasilar crackles or loud P2
Investigations
First-line
Focused clinical assessmentPattern recognition, red flags, functional impact, and targeted examination
Basic labs when indicatedCBC, CMP, ESR/CRP, CK, urinalysis, or disease-specific testing depending on suspected condition
Initial imaging when indicatedPlain radiographs or MRI/ultrasound based on suspected structural, inflammatory, infectious, or neurologic disease
Second-line
Disease-specific confirmatory testingAutoantibodies, HLA-B27, synovial fluid, nerve conduction studies, DEXA, or cultures as appropriate
MRI/ultrasound/CTUsed for early inflammatory disease, occult fracture, tendon tear, infection, or surgical planning
Screening before immunosuppressionTB, hepatitis, vaccination review, and baseline labs when biologic or high-risk therapy is planned
Specialist
Specialist referralRheumatology, orthopedics, infectious disease, ophthalmology, neurology, or spine surgery depending on red flags and disease severity
1
Initial management
- Address red flags and emergencies first
- Use guideline-directed first-line therapy matched to disease severity
- Educate the patient and set functional goals
- Use analgesia and rehabilitation when appropriate
2
Escalation
- Escalate to specialist-directed therapy if severe, refractory, progressive, or organ-threatening disease
- Use imaging, procedures, immunosuppression, antibiotics, or surgery according to diagnosis
- Monitor response objectively and revise diagnosis if response is atypical
3
Prevention and follow-up
- Manage comorbidities and medication toxicity
- Vaccinate and screen when immunosuppression is used
- Prevent disability, falls, fracture, infection, and functional decline
Complications
- Functional impairment: Pain, weakness, stiffness, deformity, or disability depending on disease
- Diagnostic delay: Missed infection, fracture, inflammatory disease, neurologic compromise, or organ-threatening complication
- Medication toxicity: NSAID, steroid, antibiotic, anticoagulation, opioid, or immunosuppressive adverse effects
- Chronic disease burden: Reduced quality of life, work impairment, deconditioning, and mental health impact
USMLE Step 2 CK Exam Tips
- 1Raynaud + skin thickening + dysphagia = systemic sclerosis
- 2Anti-centromere = limited disease/PAH risk
- 3Anti-Scl-70 = diffuse disease/ILD risk
- 4RNA polymerase III = renal crisis risk
- 5Renal crisis requires ACE inhibitor immediately
- 6Avoid high-dose steroids
practicetest your knowledge on systemic sclerosis (scleroderma)Apply what you've learnt with USMLE Step 2 CK-style questions from the iatroX Q-Bank — musculoskeletal and beyond.
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