weakness of the neck flexors, pharyngeal weakness, and diaphragmatic weakness can cause head drop, dysphagia, and respiratory compromise

predict poor outcomes

Antibodies to RNA polymerases I/III are associated with rapidly progressive diffuse skin changes and a much higher risk of renal involvement

Patients with antitopoisomerase I antibodies typically have diffuse skin changes, interstitial lung disease, and an overall worse prognosis

hypertension, proteinuria, microscopic hematuria, and thrombotic microangiopathy (schistocytes on peripheral blood smear).

Patients in the early stages of diffuse scleroderma, particularly those treated with glucocorticoids and those who produce antibodies to RNA-polymerase III are at the greatest risk for SRC

Bleeding can also occur secondary to mucosal A-V malformations or telangiectasia in the wall of the stomach (gastric antral vascular ectasia or GAVE).

pallor and cyanosis of the digits, followed by a reactive hyperemia after rewarming.

this vascular disease is fundamental to organ damage and subsequent malfunction

prominent skin changes, including marked telangiectasias (dilated capillaries)

dermal fibroblasts derived from patients with scleroderma overproduce extracellular matrix that leads to increased tissue collagen deposition in the skin

associated with pruritus and discomfort that usually lasts for weeks to months.

skin is pinched between the examiner’s thumbs in 17 specified areas of the patient’s body, scoring the thickness of the skin from 0 (normal) to 3 (very thick).

classification criteria

silica dust

50 years

Raynaud phenomenon, gastroesophageal reflux with or without dysmotility, skin changes, swollen fingers, and arthralgias.