Outcome
Type |
Measure |
Description |
Time frame |
Safety issue |
Primary |
Frequency of diaphragmatic involvement among ScS patients with dyspnea and/or with suspected respiratory muscular involvement on pulmonary function tests (PFT) |
Number of patients with dyspnea (SADOUL =2) and/or with respiratory muscular involvement suspected on PFT (maximum inspiratory pressure (max IP) below definite z-score and/or lying/sitting low vital capacity (LVC) ratio < 75%) having diaphragmatic muscular involvement confirmed by diaphragmatic electromyography (EMG) (latency >8.5ms and amplitudes <0.5mV) by neck electric phrenic stimulation and/or Pdi <20cmH2O after magnetic stimulation |
4 months maximum (maximum time between PFT realization and date to obtain EMG/Pdi) |
|
Secondary |
Sensitivity, specificity, positive and negative predictive value of pulmonary function tests compared to diaphragmatic involvement confirmed by EMG and Pdi among ScS patients with suspected pulmonary muscular involvement on PFT |
Proportion of ScS patients with diaphragmatic involvement confirmed by EMG (latency >8.5ms and amplitudes <0.5mV by neck electric phrenic stimulation and/or Pdi <20cmH2O after magnetic stimulation) compared to proportion of ScS patients with maxIP below definite z-score and/or lying/sitting LVC ratio <75% |
4 months maximum (maximum time between PFT realization and date to obtain EMG/Pdi) |
|
Secondary |
Sensitivity, specificity, positive and negative predictive value of dyspnea compared to diaphragmatic involvement confirmed by EMG and Pdi |
Proportion of ScS patients with diaphragmatic involvement confirmed by EMG (latency >8.5ms and amplitudes <0.5mV by neck electric phrenic stimulation and/or Pdi <20cmH2O after magnetic stimulation) compared to proportion of ScS patients with dyspnea (SADOUL =2) or without dyspnea |
4 months maximum (maximum time between PFT realization and date to obtain EMG/Pdi) |
|
Secondary |
Correlation between diaphragmatic involvement confirmed by EMG and and/or Pdi and dyspnea |
Proportion of ScS patients with diaphragmatic involvement confirmed by EMG (latency >8.5ms and amplitudes <0.5mV by neck electric phrenic stimulation and/or Pdi <20cmH2O after magnetic stimulation) and proportion of ScS patients with dyspnea (SADOUL =2) |
4 months maximum (maximum time between PFT realization and date to obtain EMG/Pdi) |
|
Secondary |
Correlation between diaphragmatic involvement confirmed by EMG and and/or Pdi and general and ScS-related factors |
Proportion of ScS patients with diaphragmatic involvement confirmed by EMG (latency >8.5ms and amplitudes <0.5mV by neck electric phrenic stimulation and/or Pdi <20cmH2O after magnetic stimulation) and : age in years ; proportion of female; proportion of limited cutaneous or diffuse cutaneous or sine scleroderma ; ScS duration in years, proportion of anti-centromere or anti-Scl70 or anti-ARNpolymeraseIII or "others" autoantibody; proportion of digital tip ulcerations; proportion of subcutaneous calcifications; proportion of arthritis; proportion of arterial pulmonary hypertension; proportion of interstitial lung disease; proportion of renal crisis; proportion of inflammatory myopathy; proportion of immunosuppressant use; modified Rodnan skin score (median, ranging from 0-51)); interlabial length in centimeter; proportion of telangiectasia; proportion of cardiac insufficiency (left and/or right heart); creatine phosphokinase level in UI/mL ; PO2 in mmHg; PCO2 in mmHg |
4 months maximum (maximum time between PFT realization and date to obtain EMG/Pdi) |
|
Secondary |
Correlation between diaphragmatic involvement confirmed by EMG and and/or Pdi and suspected muscular respiratory on pulmonary function test |
Proportion of ScS patients with diaphragmatic involvement confirmed by EMG (latency >8.5ms and amplitudes <0.5mV by neck electric phrenic stimulation and/or Pdi <20cmH2O after magnetic stimulation) and proportion of patients with maxIP below definite z-score and/or lying/sitting LVC ratio < 75%) |
4 months maximum (maximum time between PFT realization and date to obtain EMG/Pdi) |
|
Secondary |
Correlation between diaphragmatic involvement confirmed by EMG and and/or Pdi and quality of life and functional disability |
Proportion of ScS patients with diaphragmatic involvement confirmed by EMG (latency >8.5ms and amplitudes <0.5mV by neck electric phrenic stimulation and/or Pdi <20cmH2O after magnetic stimulation) and total score in the Functional Assessment of Chronic Illness Therapy (FACIT)-Dyspnea-short form (ranging from 0 to 30; higher values represent a worse outcome); physical summary score (mean) from the short form health survey 36 (SF-36) (French version 2.0) ranging from 0 to 100 (higher values represent a worse outcome) ; mental summary score (mean) from the short form health survey 36 (SF-36) (French version 2.0) ranging from 0 to 100 (higher values represent a worse outcome |
4 months maximum (maximum time between PFT realization and date to obtain EMG/Pdi) |
|