Systemic Sclerosis

Question 1

Subsets of systemic sclerosis or scleroderma

Answer 1

limited cutaneous SSc includes CREST syndrome (calcifications, Raynaud phenomenon, esophageal hypomotility, sclerodactyly, and telangiectasia)
diffuse cutaneous SSc
SSc without skin involvement or “systemic sclerosis sine scleroderma”

Question 2

General characteristics of scleroderma?

Answer 2

vasculopathy and fibrosis of the skin and internal organs
poor prognosis
mortality risk 2-5x higher than the general population. Most SSc-related deaths d/t complications of pulmonary fibrosis; pHTN; cardiac, renal, or gastrointestinal disease; and infections.
no cure

Question 3

Skin manifestations of scleroderma?

Answer 3

• limited: typically restricted to hands, face, and neck
• diffuse: involves chest, abdomen, forearms, upper arms, and shoulders
  Sclerodactyly
  Telangiectasias

Question 4

MSK findings of scleroderma?

Answer 4

Fibrosis around tendons and nerves, manifesting as:

• arthralgia
• tendinopathy
• myalgia
• neuropathy

Question 5

Vascular findings in scleroderma?

Answer 5

Raynaud phenomenon
Abnormalities in nail-fold capillaries (seen with a dermatoscope or other similar device)
Digital ulcers

Question 6

GI findings in scleroderma?

Answer 6

Esophageal dysmotility
Wide-mouthed diverticula
Telangiectasias
Primary biliary cirrhosis

Question 7

Renal manifestations of scleroderma?

Answer 7

Mild-to-moderate proteinuria
Increased creatinine
Hypertension
Scleroderma renal crisis

Question 8

Pulmonary findings in scleroderma?

Answer 8

Interstitial lung disease
Pulmonary arterial hypertension
Pleuritis
Endobronchial telangiectasias

Question 9

Cardiac findings in scleroderma?

Answer 9

Cardiac fibrosis
Coronary artery disease
Pericarditis

Question 10

Diagnosis of scleroderma?

Answer 10

Needs to meet 9 of criteria:
Skin thickening proximal to MCP: 9
skin thickening distal to MCP: 2-4
Fingertip lesions: 2-3
Telangiectasia: 2
Abnormal nailfold capillaries: 2
ILD or PAH: 2
Raynaud's: 3
AutoAb (anticentromere, anti Scl-70, anti RNA polymerase III): 3

Question 11

Treatment for scleroderma?

Answer 11

Skin changes: glucocorticoids, Methotrexate, Mycophenolate mofetil
MSK changes: MTX, glucocorticoids, leflunomide, surgery for nerve entrapment
Raynauds: Avoidance of exposure to the cold, Topical nitrates, CCB, Iloprost
Digital ulceration: Aspirin, Sildenafil/tadalafil, Bosentan
GI dysmotility: Metoclopramide, Erythromycin
Reflux: PPI
Renal crisis: Ramipril, Perindopril
ILD: glucocorticoids, Cyclophosphamide, Mycophenolate mofetil
PAH: Supplemental oxygen, Ambrisentan, Bosentan, Macitentan, Riociguat, Epoprostenol, Treprostinil
Rapidly progressive SSc with risk of organ failure: Autologous hemopoietic stem-cell transplantation