Systemic Sclerosis
Rheumatology · Systemic Sclerosis 🎯 Practice these cards
In the 2013 ACR/EULAR SSc classification criteria, skin thickening extending proximal to the ____ joints scores ____ points and is sufficient alone; a total score of ____ or more classifies definite SSc.
In the 2013 ACR/EULAR classification criteria, skin thickening of the fingers of both hands extending proximal to the MCP joints scores 9 points — sufficient alone — and a total score of ≥9 classifies definite systemic sclerosis.
The three SSc subtypes are ____ cutaneous SSc, ____ cutaneous SSc, and SSc ____ scleroderma.
SSc divides by skin extent into limited cutaneous SSc (LcSSc; fingers/distal limbs/face, CREST, PAH risk), diffuse cutaneous SSc (DcSSc; proximal limbs and trunk, early ILD/renal crisis), and SSc sine scleroderma (internal-organ disease without skin thickening).
Anticentromere antibodies associate with ____SSc and isolated ____; anti–Scl-70 with ____SSc and ____; anti–RNA polymerase III with ____SSc and ____.
Anticentromere → LcSSc + isolated PAH (renal crisis rare); anti–Scl-70 (topoisomerase I) → DcSSc + ILD; anti–RNA polymerase III → DcSSc + scleroderma renal crisis (+ GAVE, contemporaneous cancer).
Scleroderma renal crisis risk factors include ____ cutaneous disease, glucocorticoid doses ≥____ mg/day, and anti–____ antibodies.
SRC risk factors: diffuse cutaneous disease, glucocorticoids ≥15 mg/day, and anti–RNA polymerase III antibodies. 75% of episodes occur within 4 years of onset; incidence has fallen with glucocorticoid avoidance and CCB use for Raynaud.
SRC is treated with ____ inhibitors titrated to blood pressure and continued even if serum ____ rises, while ____ use of these drugs is not recommended.
Treat SRC with ACE inhibitors (typically captopril) titrated to BP and CONTINUE despite rising creatinine or dialysis (late recovery possible). Prophylactic ACE inhibitors are NOT recommended — they may worsen outcomes. ARBs only if ACE-intolerant.
Significant ILD occurs in about ____% of DcSSc (____% with anti–Scl-70 antibodies) and ____% of LcSSc, and is the ____ cause of disease-associated death in SSc.
Significant ILD: ~50% of DcSSc (85% with anti–Scl-70) and ~35% of LcSSc; NSIP is the most common HRCT pattern; ILD is the main cause of disease-associated death in SSc.
All SSc patients need PFT and ____ at diagnosis, then PFT with DLCO every ____ months for 5 years; a ____% FVC decline or ____% DLCO decline within 12 months signals progression.
All SSc patients: PFT + HRCT at diagnosis, then PFT with DLCO every 6–12 months for 5 years. Progression concern: ≥10% FVC decline or ≥15% DLCO decline within 12 months.
PAH affects about ____% of SSc patients; an FVC/DLCO ratio of ____ or greater suggests it, and patients should undergo ____ annually.
PAH prevalence ~10% in SSc; FVC/DLCO ratio ≥1.6 suggests PAH; screen with echocardiography annually and for new symptoms; confirm by right-heart catheterization (mPAP >20 mmHg, PCWP ≤15, PVR >2 WU).
Raynaud phenomenon occurs in about ____% of SSc patients; roughly ____% of patients with Raynaud plus an SSc autoantibody plus SSc-pattern ____ changes develop SSc.
Raynaud phenomenon occurs in ~95% of SSc patients and is usually the earliest manifestation. ~80% of patients with Raynaud + an SSc autoantibody + SSc-pattern nailfold capillary changes develop SSc.
MCTD is defined by antibodies to ____ and overlap features of SSc, myositis, or SLE; its major cause of death is ____.
MCTD = overlap features of SSc/myositis/SLE PLUS anti-U1-RNP (70-kD epitope) antibodies; PAH is the major cause of death; anti-dsDNA/anti-Smith/anti-Ro favor primary SLE instead. UCTD evolves into a definite disease (most often SLE) within 2–5 years in a minority.
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