Systemic Sclerosis

Question · easy

A 34-year-old woman reports a 3-year history of cold-induced color changes in her fingers and now presents with puffy hands, gastroesophageal reflux, and facial telangiectasias. ANA testing is positive. Which of the following is the most common and typically the earliest clinical manifestation of systemic sclerosis?

Sclerodactyly Esophageal dysmotility Raynaud phenomenon Digital tip ulcers

Question · medium

A 41-year-old woman with rapidly progressive diffuse skin thickening is found to have a new autoantibody. Which autoantibody most strongly predicts the development of scleroderma renal crisis?

Anti-U1-RNP Anti–Scl-70 (anti–topoisomerase I) Anticentromere antibody Anti–RNA polymerase III

Question · hard

A 45-year-old man with diffuse cutaneous systemic sclerosis presents with headache and blurred vision; blood pressure is 212/124 mmHg, creatinine 3.4 mg/dL (baseline 0.9), and smear shows microangiopathic hemolytic anemia. Which management principle is correct?

An angiotensin receptor blocker is preferred over an ACE inhibitor as first-line therapy Prophylactic ACE inhibition should have been started earlier; it is now contraindicated Plasma exchange should replace antihypertensive therapy as the primary intervention Start an ACE inhibitor, titrate to blood pressure, and continue it even if creatinine rises further or dialysis is needed

Question · hard

A 38-year-old woman with diffuse cutaneous systemic sclerosis and biopsy-proven NSIP-pattern interstitial lung disease is started on mycophenolate mofetil. Which statement best reflects evidence-based management of SSc-associated ILD?

Pirfenidone is strongly recommended as first-line antifibrotic therapy for SSc-ILD Nintedanib improves lung function and also reverses skin and gastrointestinal manifestations A 5% decline in FVC over 12 months meets the threshold for definite progression Mycophenolate and cyclophosphamide produce similar FVC outcomes, but mycophenolate is better tolerated and has become the mainstay of therapy

Question · medium

A 28-year-old woman has puffy fingers, Raynaud phenomenon, nonerosive inflammatory arthritis, and esophageal dysmotility. ANA shows a high-titer speckled pattern. Which autoantibody defines her most likely diagnosis, and what is its major cause of death?

Anti–double-stranded DNA; lupus nephritis Anti–Scl-70; interstitial lung disease Anti–Jo-1; antisynthetase interstitial lung disease Anti-U1-RNP; pulmonary arterial hypertension