SSc 101. Julia Rhiannon June 2013 j email@example.com. outline. Intro and pathogenesis Classification dcSSc ~ diffuse cutaneous systemic sclerosis organ involvement with 2 cases Rodnan skin scoring lcSSc ~ limited cutaneous systemic sclerosis & case
Coup de Sabre
The next step would be:
1. Check for nailfold changes
2. Doppler study
3. Order ANA panel
4. Order ANCA
5. Skin biopsy
6. Pred 60mg IM x1
7. Tell her to take her
Scleroderma renal crisis/malignant Htn
arteriolar fibrinoid necrosis
FootClinical Assessment of Skin Thickening-Modified Rodnan Skin Score (MRSS)
HPI - A 52 y/o women with 10 yrs of Raynaud’shere w/ recently increasing problems with hands as well as dysphagia. She has also noted small red dots on her face and hands appearing over the last 6 months.
Exam - In the office she has active Raynaud’sand has multiple small red lesions on face, hands, and tongue.
What pattern would you expect
to see on ANA testing?
Arthritis Rheum. 2009 Apr;60(4):1112-28.
Steen VD. Autoantibodies in systemic sclerosis. Semin Arthritis Rheum 35:35-42, 2005.
Toxic oil syndrome- Spain - rapeseed oil contaminated with aniline dye SScl like disease
contaminated L-tryprophan - 1989 – “eosinophiliamyalgiasyndrome”
Silica dust - case reports in miners
Organic solvents- textile industry / paint thinners and removers
Arch Occup Environ Health 2004; 77:10-16
1.6% of Ssc pts have a 1st degree relative with SSc (RR=13)
5.9% concordance in identical twins (300 times higher than chance)
Strong associations with HLA-A1, -B8, -DR3 haplotype and with DR3/DR52
Strong associations with C4AQ0 and DQA2
Well-defined cluster of Choctaw Indians with diffuse phenotypeEnv/Occup Genetics
“No drug has been shown to be completely worthless until tried in Scleroderma” –Eric Bywaters
(1012 Italian patients)