Scleroderma Flashcards
(33 cards)
What is Scleroderma?
It is a connective tissue disorder characterized by skin thickening and tightening (hardening of the skin)
What are the two major types of scleroderma?
Localized and Systemic (Systemic sclerosis).
What is morphea?
A form of localized scleroderma with thickened patches of skin.
What is βen coup de sabreβ?
Linear scleroderma affecting one side of the face or scalp.
What does systemic sclerosis affect?
Both skin and internal organs.
What is the CREST syndrome?
Limited cutaneous systemic sclerosis: Calcinosis, Raynaudβs, Esophageal dysmotility, Sclerodactyly, Telangiectasia.
Systemic Scleroderma is divided into?
Limited cutaneous disease a.k.a Crest Syndrome
Diffuse cutaneous disease
Who is most affected by scleroderma?
Women aged 30β50, especially those of African descent.
What is the major cause of morbidity in scleroderma?
Pulmonary complications like PAH and ILD.
What causes fibrosis in scleroderma?
Autoimmune activation of fibroblasts leading to excessive collagen production.
What triggers endothelial injury in scleroderma?
Vasculopathy and inflammation.
What does endothelial injury lead to?
Hypoxia, cytokine release, and progressive fibrosis.
What is Raynaudβs phenomenon?
It is an exaggerated vascular reaction in response to cold temperatures or emotional stress.
Triphasic color change in fingers: white (due to lack of blood flow) β blue (due to O2 depletion in tissues) β red (As blood flow returns).
What skin changes are seen in scleroderma?
Thickening
Salt and pepperβ appearance
Ulcers
Edematous Skin
Reduced oral aperture.
What GI symptoms are common in scleroderma?
GERD
Dysphagia
Dry mouth
Dyspepsia
GAVE (gastric antral vascular ectasia).
What are the main lung complications in scleroderma?
PAH in limited cutaneous, ILD in diffuse cutaneous.
What is scleroderma renal crisis?
H- Hypertension (arterial)
A- Acute hypertensive retinopathy (grade 3/4)
R- Renal failure
M- MAHA.
Which antibody is linked to PAH in limited cutaneous scleroderma?
Anti-centromere antibody.
Which antibody is associated with ILD in diffuse cutaneous scleroderma?
Anti-Scl-70 or Anti-topoisomerase I
Which antibody indicates risk for renal crisis?
Anti-RNA polymerase III.
What imaging is used to assess ILD in scleroderma?
High-resolution CT (HRCT).
What test assesses pulmonary function in scleroderma?
Pulmonary function test (PFT) and Diffusing Capacity For Carbon Monoxide (DLCO).
How is skin managed in scleroderma?
Use emollients (moisturizer) and low-dose corticosteroids.
Why avoid high-dose steroids in scleroderma?
They increase the risk of scleroderma renal crisis.
