Table 1 Appropriate criteria for the definition of disease relapse in patients with rapidly progressive SSc treated with AHSCT.

From: Definition of relapse criteria in patients with rapidly progressive systemic sclerosis treated with autologous haemopoietic stem cell transplantation

Appropriate criteria for the definition of disease relapsea

A. With strong agreement

Skin

 1. 25% increase in mRSS

 Definition: Assessment of skin thickness on a scale from 0 (normal thickness) to 3 + (severe thickness) at 17 anatomic areas (values from 0–51).

Lung

 2. Decrease of lung function tests: ≥10% in FVC and/or ≥15% in DLCO of the predicted values.

 3. Increase of interstitial lung disease (assessed though HRCT) defined by an expert radiologist

 4. Both decrease lung function tests and increase of interstitial lung disease.

Heart

 5. Congestive heart failure

 Definition: non-systemic sclerosis related causes must have been reasonably excluded by an experienced cardiologist

Kidney

 6. New appearance or worsening of renal involvement

 Definition: Urine analysis abnormalities (proteinuria, hematuria, casts), new/worsening of renal insufficiency (serum creatinine > upper limit of normal/rise > 25%) with or without accelerated/ malignant hypertension.

Death

 7. Related to the disease

B. With fair agreement

Tendons

 1. New appearance or worsening of tendon rubs

 Definition: Perception of leathery crepitus during motion of hands, wrists, elbows, shoulders, knees and ankles (both at anterior and posterior aspects)

Lung

 2. New appearance of pulmonary hypertension

 Definition: resting mean pulmonary artery pressure (mPAP) > 20 mmHg (by right heart catheterization)

Joints

 3. New appearance or worsening of articular involvement

 Definition: symmetric swelling and tenderness of the peripheral joints

Muscle

 4. New or worsening in muscle involvement

 Definition: as detected at physical examination associated with CK increase

Weight loss

 5. ≥ 15% unvoluntary drop in body weight

Systemic

 6. End stage organ failure

  1. aThe listed criteria should be strictly related to the course of SSc. The possibility that the appearance or worsening of any feature may be caused by other pathologic conditions should be excluded by an expert physician of the specific subspecialty.
  2. mRSS modified Rodnan Skin Score, FVC Forced Vital Capacity, DLCO Diffusing capacity of the Lungs for Carbon monoxide, HRCT High-Resolution Computed Tomography, mPAP mean Pulmonary Artery Pressure, CK Creatine Kinase.
Back to article page