HARD SKIN, HARD TRUTHS
This form of scleroderma affects the skin without internal organ involvement.
What is localized scleroderma
This historical acronym for calcinosis, Raynaud’s, esophageal dysmotility, sclerodactyly, and telangiectasia is now considered outdated.
What is CREST
This autoantibody is most commonly associated with limited cutaneous systemic sclerosis
What is anticentromere antibody
A DLco less than 60% with minimal ILD strongly suggests this life-threatening complication.
What is pulmonary arterial hypertension
This medication class is first-line therapy for Raynaud phenomenon in systemic sclerosis.
What are calcium channel blockers
Morphea and this subtype that may present as “en coup de sabre” are the two main forms of localized scleroderma.
What is linear scleroderma
A localized scleroderma variant with dermal fibrosis but no systemic involvement; usually limited to plaques on the trunk or limbs.
Morphea
This autoantibody confers the highest risk of scleroderma renal crisis.
What is anti–RNA polymerase III
This is the gold-standard diagnostic test required to confirm pulmonary arterial hypertension.
What is right heart catheterization
This immunosuppressive agent is preferred for long-term treatment of progressive SSc-associated ILD due to better tolerability.
What is mycophenolate mofetil
Among SSc subsets, this one has the strongest association with late-onset pulmonary arterial hypertension.
What is limited cutaneous systemic sclerosis
Skin thickening confined to face, neck, and distal extremities; often associated with anticentromere antibodies.
What is Limited cutaneous systemic sclerosis
Data suggests that patients with this positive antibody are at higher risk for malignancy
RNA polymerase III antibody ?
This renal complication classically presents with acute hypertension and thrombotic microangiopathy.
What is scleroderma renal crisis
Despite improving outcomes once renal crisis occurs, this medication class has not been shown to prevent scleroderma renal crisis when used before onset.
What are ACE inhibitors
This clinical feature carries the highest individual weighted score in the 2013 ACR/EULAR systemic sclerosis classification criteria.
What is skin thickening of the fingers of both hands extending proximal to the MCP joints?
This subtype describes patients with internal organ involvement and SSc-specific autoantibodies but no skin thickening.
What is systemic sclerosis sine scleroderma
An isolated nucleolar ANA pattern often corresponds to these antibodies and increased ILD risk.
What are anti–U3 RNP or anti–Th/To antibodies
The most common histopathologic pattern of ILD seen in systemic sclerosis is this fibrotic lung process.
What is nonspecific interstitial pneumonia
This antifibrotic agent slows decline in FVC in patients with SSc-associated ILD without improving extrapulmonary disease.
What is nintedanib
This early microvascular abnormality underlies Raynaud phenomenon and precedes overt fibrosis in SSc.
What is endothelial dysfunction with capillary dropout?
Overlap syndrome refers to
SSc features coexist with another connective tissue disease (e.g., myositis, RA, or lupus), often complicating management and prognosis.
The presence of any of these three antibodies contributes three points toward classification in the 2013 ACR/EULAR criteria.
What are anticentromere, anti–Scl-70, or anti–RNA polymerase III antibodies
This GI vascular lesion, also called “?” can cause chronic iron deficiency anemia.
What is gastric antral vascular ectasia "watermelon stomach"
This therapy improves survival in severe early diffuse SSc but carries a 3–10% treatment-related mortality risk.
What is autologous hematopoietic stem cell transplantation