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BSR Guidelines for Systemic Sclerosis Management

d Jack Cush, MD

The British Society for Rheumatology (BSR) has updated its 2015 guidelines for the management of patients with sytemic sclerosis (SSc) based on published evidence, systematic literature review and expert opinion. 

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Cross-sectional study 69 Systemic Sclerosis Pts: 33% w/ PPI-refractory esophagitis & 2/3 w/ EGD normal esophageal mucosa. PPI-refractory esophagitis was more commonly linked to diffuse SSc subset (43 vs 17%) and GI dysmotility (66 vs 8%; p = <0.001) https://t.co/yIBlyNZacB https://t.co/dempwjX5XO

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Systemic Sclerosis Insights (9.13.2024)

d Jack Cush, MD

Dr. Jack Cush reviews the news and journal articles from this past week on RheumNow.com.

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Morphea,crop

Increasing Incidence of Morphea

d Jack Cush, MD

A large EMR derived cohort study of over 10,000 patients with morphea (localized scleroderma) sheds light on its prevalence and associated features.

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Systematic literature review IVIG in systemic sclerosis included 12 studies, 266 pts. A favorable effect was seen for skin thickening, MSK pain, GI Sxs, Steroid use, QOL. But less effect for pulmonary Dz, PFTs (stabilized?). Studies were not of high-quality… https://t.co/c3Xix9K2XK https://t.co/L3LKwegmkf
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While Raynaud's phenomenon severity is worst at very cold temperatures, it may also worsen during very warm temperatures, presumably due to air conditioning. Based on q3mos assessments of 2243 RP pts (88% female, 82% White, mean 55 yrs https://t.co/4c0XzqHYjB https://t.co/TOn4DJP4Xi

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"Vanishing Fingers" Acro-osteolysis in a patient with Scleroderma and renal failure and bilateral hand pain. Causes of AO include Scleroderma, PsA, hypertrophic osteoarthropathy, skeletal dysplasias, frostbite, Hyperparathyroidism, genetic disorders https://t.co/UwXcw3r6LD https://t.co/bnGiY0i0ot

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Linger on the Fingers (9.6.2024)

d Jack Cush, MD

Dr. Jack Cush reviews the news and journal reports from the past week on RheumNow.com. This week with a focus on fingers and better prescriptive follow through.

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Scleroderma Raynaud's May Worsen in Summer

d MedPage Today

Episodes of Raynaud's phenomenon, a common feature of systemic sclerosis (SSc, also known as scleroderma), were more severe not only at low temperatures, as one would expect, but also during summertime heat, a multinational study found.

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Scleroderma skin findings in pts w/ skin of colour (SOC) - Raynauds was common but SOC pts had fewer hallmark Sxs: telangiectasias, calcinosis, digital swelling (complicating Dx). SOC had more severe pulmonary Dz & increased mortality, Atypical skin findings… https://t.co/8XkelxqvYE https://t.co/JOw8OSNjzw
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Running with Data (8.30.2024)

d Jack Cush, MD

Dr. Jack Cush reviews the news and journal reports from the past week on RheumNow.com - including tips on steroids, MAS, and myositis testing.

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Cohort study shows GERD in 1539/1632 (94%) #SSc patients & 450/469 (96%) of SSc-ILD pts. GERD didnt influence ILD Dx or severity. But GERD Rx (PPI, H2RA) assoc w/ improved survival in those Scleroderma pts with ILD (p = 0.002) https://t.co/stQkpbmBCP https://t.co/e4BBz5c0av

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Causes of Carpal Tunnel Syndr: obesity, repetitive wrist movements, pregnancy, RA, PsA. but also Sjögren's syndrome, Behcet's disease and systemic sclerosis (weaker) https://t.co/ssrs8UJEpX

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2024 Update of EULAR recommendations for the Treatment of Systemic Sclerosis https://t.co/52XEfgVCzZ https://t.co/d1zgTYUeOo

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Higher Cancer Rates (8.16.2024)

d Jack Cush, MD

Dr. Jack Cush reviews the news and journal reports from the past week on RheumNow.com, noting his concerns about diet, cancer, ILD, death and dosing challenges.

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Depends on Where You’re Looking (8.9.2024)

d Jack Cush, MD

Dr. Jack Cush reviews the news and journal reports from RheumNow.com this week.

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EU study of 1052 systemic sclerosis pts failed to show early immunosuppressive use (vs late) lowered ILD risk. Early Rx (Dz Dur < 3yrs) group (n=547, 52%) more male, diffuse Dz, 60% Rx w/ MTX (1.7% biologics); ILD in 47% after3.6yrs (HR 1.13;0.93–1.38) https://t.co/PtQlgyX0TE https://t.co/R7X3zvPvIg

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NEJM Clinical Images: Coup de Sabre (linear scleroderma) 70-year-old woman with a history of hypothyroidism presented to the dermatology clinic with a 1-year history of progressive indentation and darkening of the skin of her forehead https://t.co/RiVNQiy5MM https://t.co/pxVEqBIUWP

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Calcinosis cutis affects 40% of SSc pts w/ significant morbidity. Assoc w/ Long Dz duration, vascular dysfunction, & osteoporosis. Dx by radiography. Surgical treatment is most effective therapy, but no medical therapies have proven efficacy in RCTs https://t.co/ocQ5okBauq https://t.co/1DUm6FKfm8

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Lead with Lupus (8.2.2024)

d Jack Cush, MD

Dr. Jack Cush reviews the news and journal reports from this past week on RheumNow.com - leading with lupus because everyone wants to know about lupus!

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