Scleroderma 5% Flashcards
What toxins have been linked to scleroderma-like disease?
Polyvinyl chloride, bleomycin, pentazocine, contaminated rapeseed oil, paraffin or silicone, cocaine, taxanes (paclitaxel).
What race/ethnicity has a higher frequency of scleroderma?
African American and Choctaw Native Americans
The most severe phenotypes are in AA women with younger age of onset
What is the typical demographic for juvenile systemic sclerosis
8-10.5-year-old female > male (4 female > 1 male) with Caucasian predominance
In patients with overlap syndrome with a systemic sclerosis phenotype, what other rheumatologic condition does it typically overlap with?
Overlap syndrome with dermatomyositis.
Children often present with Raynaud sclerodactyly, Gottron papules, nailfold capillary changes, myositis and arthritis
⅓ of kids present with overlap disease - Ku, PM-Scl and U1-RNP Ab’s more common in children because of this
Children lack anti-centromere ab, especially if <10 years at onset (true even for children with lcSSc)
What percentage of patients with systemic sclerosis will have a first-degree relative with autoimmune disease?
15-20%
Familial occurrences of juvenile systemic sclerosis is rare
What condition may present with a “claw hand deformity”?
Systemic sclerosis from sclerodactyly and loss of ROM in joints
What condition can cause acro-osteolysis where you get resorption of distal phalanges?
Systemic sclerosis
What factors are associated with the best outcomes in systemic sclerosis?
Early diagnosis
Early treatment
Avoiding smoking
What are the standard treatments for diffuse skin involvement in systemic sclerosis, especially within the first 3 years of disease onset?
Methotrexate - if also have arthritis and myositis
Mycophenolate - if also have ILD
What are some things that have been associated with the development of eosinophilic fasciitis?
L-tryptophan
Borrelia burgdorferi infection
Strenuous exercise
What are classic labs abnormalities seen with eosinophilic fasciitis?
Peripheral eosinophilia
High IgG
Elevated aldolase
May have high WBC, low Hgb, high plt
What are classic MRI and skin biopsy findings in eosinophilic fasciitis?
MRI: edema and thickening of involved fascia
Skin biopsy: full thickness skin biopsy shows thickening of fascia with inflammatory infiltrate consisting of lymphocytes, plasma cells, histiocytes, and eosinophils
What is the treatment for eosinophilic fasciitis?
Steroids
With or without methotrexate
A patient presents with indurated skin and thickening of her lower leg. A full thickness biopsy of the skin and soft tissues overlying indurated tender skin shows intense inflammatory infiltrate in the dermis only. What type of scleroderma is this biopsy describing?
Superficial circumscribed morphea. Other forms of linear scleroderma may involve deeper layers of the SQ tissue.
You suspect linear scleroderma that is active and present for at least 6 months…
Of the following, the MOST useful next test in confirming the suspected diagnosis is
A.laboratory assessment for ß-hemolytic streptococcal infection
B.magnetic resonance imaging to assess for fascial inflammation
C.radiography to assess for erosive joint damage
D.skin biopsy to evaluate for thickened collagen bundles
D.skin biopsy to evaluate for thickened collagen bundles
While the diagnosis of linear scleroderma is often made based on clinical findings, a skin biopsy may assist in providing confirmation.
Pathologic evaluation initially reveals inflammatory infiltrates consisting of lymphocytes, plasma cells, macrophages, eosinophils, and mast cells. Over time, there is increased accumulation of fibroblasts and collagen.
