Scleroderma renal crisis
Scleroderma renal crisis is an abrupt, organ-threatening emergency in systemic sclerosis marked by severe new-onset hypertension, acute kidney injury, and microangiopathic hemolytic anemia with schistocytes, occurring most often within the first 4 years of diffuse cutaneous disease in patients with anti-RNA polymerase III antibodies or recent high-dose glucocorticoid exposure. Treatment is immediate, aggressive titration of a short-acting angiotensin-converting enzyme inhibitor, typically captopril, continued even through a rising creatinine or dialysis.
- 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: scleroderma renal crisis is an abrupt, organ-threatening complication of systemic sclerosis (SSc) defined by sudden severe hypertension together with acute kidney injury (AKI), most often…
- Epidemiology
Incidence by subtype: affects about 5% of patients with diffuse cutaneous SSc (dcSSc) and about 2% of patients with limited cutaneous SSc (lcSSc); some series report an overall incidence closer to 10% across all SSc.
- Risk factors
Diffuse cutaneous disease: dcSSc carries a far higher risk than lcSSc (5% versus 2%), especially with rapidly progressive skin thickening in the first several years.
- Pathophysiology
Vascular injury: endothelial injury in the renal microvasculature leads to vascular constriction, intimal thickening, and fibrin deposition in the afferent arterioles, with endothelin-1 playing a central role.
- Clinical features
New, severe hypertension: abrupt onset in a patient with known or unrecognized SSc, often with a marked change from the patient's own ambulatory baseline blood pressure.
- Differential diagnosis
Thrombotic thrombocytopenic purpura (TTP): shares MAHA, thrombocytopenia, and AKI, but ADAMTS13 activity below 10% supports TTP while activity is normal in scleroderma renal crisis, and the blood…
- Diagnosis
What establishes the diagnosis: scleroderma renal crisis is a clinical diagnosis, not one defined by fixed numeric criteria; it is recognized by abrupt severe hypertension (often a marked rise from…
- Management
Start an ACE inhibitor immediately on clinical suspicion; do not delay for confirmatory testing.
- Complications
Chronic or end-stage kidney disease: patients who require dialysis may remain dialysis-dependent if renal recovery is incomplete.
- Prognosis
ACE inhibitors transformed outcomes: mortality was very high before ACE inhibitor therapy became standard; captopril and other ACE inhibitors can be lifesaving and extend life expectancy when started promptly.
- Prevention
Do not use prophylactic ACE inhibitors in asymptomatic high-risk patients; prophylactic use does not prevent scleroderma renal crisis and may worsen renal outcomes, so it is not recommended even with…
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.
- Thrombotic thrombocytopenic purpura (TTP)
Thrombotic thrombocytopenic purpura is a thrombotic microangiopathy caused by severe ADAMTS13 deficiency that lets ultralarge von Willebrand factor multimers drive platelet microthrombi; the key dyad of microangiopathic hemolytic anemia plus thrombocytopenia without another cause mandates the PLASMIC score, an ADAMTS13 activity and inhibitor level, and emergent plasma exchange started before results return.
- Hemolytic uremic syndrome
Hemolytic uremic syndrome is a thrombotic microangiopathy defined by the triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury, most often triggered in children by Shiga toxin-producing Escherichia coli O157:H7 after bloody diarrhea. Diagnosis rests on schistocytes with a negative Coombs test plus a normal ADAMTS13 level to exclude TTP, and typical STEC-HUS is managed supportively while antibiotics and antimotility agents are avoided, whereas complement-mediated atypical HUS requires eculizumab.
- Antiphospholipid syndrome
Antiphospholipid syndrome is an acquired autoimmune thrombophilia in which antiphospholipid antibodies cause recurrent venous and arterial thrombosis and pregnancy morbidity, paradoxically prolonging the aPTT in vitro while promoting clotting in vivo. Diagnosis requires a qualifying clinical event plus a laboratory antibody confirmed positive on two occasions at least 12 weeks apart, and thrombotic disease is treated with indefinite warfarin rather than a DOAC.
- Acute kidney injury
Acute kidney injury is a sudden rise in serum creatinine or fall in urine output from prerenal, intrinsic, or postrenal causes, defined and staged by KDIGO criteria. Management targets the underlying cause, corrects volume and electrolyte derangements, and escalates to renal replacement therapy for refractory hyperkalemia, acidosis, volume overload, or uremia.