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Musculoskeletal - Systemic Sclerosis (Scleroderma)
In this episode, we review systemic sclerosis (scleroderma), a rare connective tissue disease characterized by autoimmune endothelial injury and progressive fibrosis of the skin and internal organs. We break down hallmark features such as Raynaud phenomenon, skin tightening, GERD, and dyspnea, along with key physical exam findings like sclerodactyly and telangiectasias. The discussion highlights important autoantibodies—including anti–Scl-70 and anticentromere antibodies—plus essential screening for pulmonary hypertension and interstitial lung disease.
References
1) Bushardt RL, Colomb-Lippa DM, Klinger AM, Reed H. The JAAPA QRS Review for PAs: Study Plan and Guide for PANCE and PANRE. 1st ed. LWW; 2021. ISBN: 9781975143817.
2) Volkmann ER, Andréasson K, Smith V. Systemic sclerosis. Lancet. 2023 Jan 28;401(10373):304-318. doi: 10.1016/S0140-6736(22)01692-0. Epub 2022 Nov 25. PMID: 36442487; PMCID: PMC9892343.
3) Jerjen R, Nikpour M, Krieg T, Denton CP, Saracino AM. Systemic sclerosis in adults. Part I: Clinical features and pathogenesis. J Am Acad Dermatol. 2022;87(5):S0190-9622(22)00190-6. doi:10.1016/j.jaad.2021.10.065.
4) Jerjen R, Nikpour M, Krieg T, Denton CP, Saracino AM. Systemic sclerosis in adults. Part II: management and therapeutics. J Am Acad Dermatol. 2022 Nov;87(5):957-978. doi: 10.1016/j.jaad.2021.10.066. Epub 2022 Feb 4. PMID: 35131401.
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By Kristopher Maday, PA-C, DFAAPAIn this episode, we review systemic sclerosis (scleroderma), a rare connective tissue disease characterized by autoimmune endothelial injury and progressive fibrosis of the skin and internal organs. We break down hallmark features such as Raynaud phenomenon, skin tightening, GERD, and dyspnea, along with key physical exam findings like sclerodactyly and telangiectasias. The discussion highlights important autoantibodies—including anti–Scl-70 and anticentromere antibodies—plus essential screening for pulmonary hypertension and interstitial lung disease.
References
1) Bushardt RL, Colomb-Lippa DM, Klinger AM, Reed H. The JAAPA QRS Review for PAs: Study Plan and Guide for PANCE and PANRE. 1st ed. LWW; 2021. ISBN: 9781975143817.
2) Volkmann ER, Andréasson K, Smith V. Systemic sclerosis. Lancet. 2023 Jan 28;401(10373):304-318. doi: 10.1016/S0140-6736(22)01692-0. Epub 2022 Nov 25. PMID: 36442487; PMCID: PMC9892343.
3) Jerjen R, Nikpour M, Krieg T, Denton CP, Saracino AM. Systemic sclerosis in adults. Part I: Clinical features and pathogenesis. J Am Acad Dermatol. 2022;87(5):S0190-9622(22)00190-6. doi:10.1016/j.jaad.2021.10.065.
4) Jerjen R, Nikpour M, Krieg T, Denton CP, Saracino AM. Systemic sclerosis in adults. Part II: management and therapeutics. J Am Acad Dermatol. 2022 Nov;87(5):957-978. doi: 10.1016/j.jaad.2021.10.066. Epub 2022 Feb 4. PMID: 35131401.