Msk Dermatology · Year 2 · from Msk Dermatology
Case 2: Diffuse Cutaneous Systemic Sclerosis with Renal Crisis
Patient Presentation
Demographics: 52-year-old Caucasian woman
Chief Complaint: "My skin has been getting tight all over, and now I have a terrible headache and my vision is blurry."
History of Present Illness: The patient was diagnosed with systemic sclerosis 18 months ago when she developed Raynaud phenomenon and skin thickening of her fingers. Over the past year, her skin tightening has progressed to involve her arms, chest, and face. She has had worsening gastroesophageal reflux and difficulty swallowing solids. She was recently started on prednisone 25 mg daily by her rheumatologist for progressive skin disease. She now presents with acute onset of severe headache, blurred vision, and malaise beginning 24 hours ago.
Past Medical History:
- Systemic sclerosis (diffuse cutaneous) diagnosed 18 months ago
- Raynaud phenomenon
- GERD
- No prior hypertension
Medications:
- Prednisone 25 mg daily (started 3 weeks ago)
- Omeprazole 40 mg twice daily
- Nifedipine 30 mg daily for Raynaud
Allergies: NKDA
Physical Examination
- Vital Signs: BP 218/134 mmHg, HR 108 bpm, RR 22, Temp 36.9C
- General: Appears ill, anxious
- HEENT: Retinal hemorrhages and exudates on fundoscopy (hypertensive retinopathy)
- Skin:
- Diffuse skin thickening involving fingers, hands, forearms, upper arms, face, and anterior chest
- Sclerodactyly with tapered fingers
- Digital pitting scars
- Telangiectasias on face and hands
- Skin score 32/51 (modified Rodnan skin score)
- Cardiovascular: Tachycardia, regular rhythm, S4 gallop
- Lungs: Clear to auscultation
- Abdomen: Non-tender
Workup and Results
Laboratory Studies:
| Test | Result | Reference Range |
|---|---|---|
| Creatinine | 4.2 mg/dL | 0.6-1.2 mg/dL (baseline 0.9 mg/dL) |
| BUN | 68 mg/dL | 7-20 mg/dL |
| Hemoglobin | 8.4 g/dL | 12-16 g/dL |
| Platelets | 72,000/mm3 | 150,000-400,000/mm3 |
| LDH | 780 U/L | 140-280 U/L |
| Haptoglobin | <10 mg/dL | 30-200 mg/dL |
| Reticulocyte count | 4.8% | 0.5-2.5% |
| Peripheral smear | Schistocytes present | None |
| ANA | 1:320 | <1:80 |
| Anti-Scl-70 | Negative | Negative |
| Anti-RNA polymerase III | Positive | Negative |
| Urinalysis | Protein 2+, RBC 25/hpf | Normal |
Imaging:
- Chest X-ray: Normal cardiac silhouette
- Renal Ultrasound: Normal-sized kidneys without obstruction
Clinical Image
Clinical photograph demonstrating sclerodactyly in systemic sclerosis with tight, shiny skin of the fingers, tapered digits, and flexion contractures. Source: Wikimedia Commons, CC BY-SA 4.0.
Diagnosis
Scleroderma Renal Crisis with Microangiopathic Hemolytic Anemia
Diagnostic Features:
- New-onset severe hypertension (previously normotensive)
- Rapidly progressive acute kidney injury
- Microangiopathic hemolytic anemia (schistocytes, elevated LDH, low haptoglobin)
- Thrombocytopenia
- Diffuse cutaneous systemic sclerosis within first 4 years
- Positive anti-RNA polymerase III antibodies (strongly associated with renal crisis)
- Recent high-dose corticosteroid use (precipitating factor)
Treatment Plan
Emergent Management:
- ACE inhibitor - CRITICAL: Captopril 6.25 mg initially, titrate rapidly to control BP
- ACE inhibitors are lifesaving in scleroderma renal crisis
- Continue even if creatinine rises initially
- Target BP <140/90 within 72 hours
- Admit to ICU for close monitoring
- Discontinue prednisone - steroids may have precipitated crisis
- Prepare for possible dialysis - may be needed temporarily
Blood Pressure Management:
- ACE inhibitors are first-line and should not be discontinued
- Add additional agents (calcium channel blockers) if needed
- Avoid beta-blockers initially (may worsen Raynaud)
Monitoring:
- Hourly blood pressure initially
- Daily creatinine, LDH, hemoglobin, platelets
- Urine output
Prognosis Discussion:
- 1-year survival has improved to ~80% with ACE inhibitors (was <15% before)
- ~50% may require temporary dialysis
- ~20-30% may recover renal function and come off dialysis
Teaching Points
- Scleroderma renal crisis: Medical emergency characterized by acute hypertension and AKI; occurs predominantly in early diffuse disease
- Anti-RNA polymerase III antibodies: Strongly associated with renal crisis (and malignancy); presence should heighten vigilance
- Corticosteroids as trigger: Doses >15 mg prednisone/day increase risk of renal crisis; avoid high-dose steroids in diffuse scleroderma
- ACE inhibitors are critical: Continue even if creatinine rises; they have transformed outcomes in this previously fatal complication
- Microangiopathic hemolytic anemia: Schistocytes, elevated LDH, low haptoglobin, thrombocytopenia - results from endothelial damage in renal vasculature