Criteria for Rapidly (Fulminant) Progressive Systemic Sclerosis

Criteria for rapidly progressive (fulminant) PSS:

(1) presence of PSS

(2) one or more organ failures within 3 years after the onset of the initial symptoms

Criteria for respiratory failure - both of the following:

(1) exertional dyspnea or effort intolerance

(2) one of the following:

(2a) pulmonary hypertension with cor pulmonale

(2b) interstitial fibrosis with impaired carbon monoxide diffusion (< 50% of predicted)

(2c) resting hypoxemia (PaO2 < 60 mm Hg) AND hypercapnia (PaCO2 > 49 mm Hg)

Criteria for renal failure - one or both of the following:

(1) creatinine clearance < 10 mL per minute

(2) oliguria AND serum creatinine >= 2.0 mg/dL

Criteria for cardiac failure:

(1) presence of congestive heart failure

In the implementation an organ failure with onset before symptoms was not counted.