Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Dyspnea in systemic sclerosis (ScS) constitute a major factor of functional disability.
Intensity of dyspnea is sometimes discordant with objectives data from cardiopulmonary involvements, suggesting unknown additional factors.
Diffuse fibrosing myopathy of bad prognosis have been reported in ScS.To now, muscular respiratory involvement has not been evaluated in ScS. Therefore, ScS patients (with or without dyspnea) could have underlying respiratory muscular involvement not detected by current standard of care with pulmonary function tests (PFT).
This project is the first, to the best of our knowledge, to assess frequency of respiratory muscular involvement in ScS and to evaluate a screening strategy of this involvement.
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Muscular explorations | Experimental | Pulmonary function test and diaphragmatic electromyography |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Pulmonary function test | Diagnostic Test | Diaphragmatic electromyography with measurement of transdiaphragmatic pressure in cases of dyspnea and/or abnormal pulmonary function test (maximum inspiratory pressure and/or lying/seating low vital capacity ratio) |
| Measure | Description | Time Frame |
|---|---|---|
| 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) |
| Measure | Description | Time Frame |
|---|---|---|
| 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% |
Not provided
Inclusion Criteria:
Exclusion Criteria:
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Chu de Poitiers | Poitiers | France |
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
| ID | Term |
|---|---|
| D012595 | Scleroderma, Systemic |
| D012131 | Respiratory Insufficiency |
| D018908 | Muscle Weakness |
| ID | Term |
|---|---|
| D003240 | Connective Tissue Diseases |
| D017437 | Skin and Connective Tissue Diseases |
| D012871 | Skin Diseases |
| D012120 | Respiration Disorders |
Not provided
Not provided
| ID | Term |
|---|---|
| D012129 | Respiratory Function Tests |
| ID | Term |
|---|---|
| D003948 | Diagnostic Techniques, Respiratory System |
| D019937 | Diagnostic Techniques and Procedures |
| D003933 | Diagnosis |
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
| 4 months maximum (maximum time between PFT realization and date to obtain EMG/Pdi) |
| 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) |
| 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) |
| 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) |
| 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) |
| 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) |
| D012140 | Respiratory Tract Diseases |
| D009135 | Muscular Diseases |
| D009140 | Musculoskeletal Diseases |
| D020879 | Neuromuscular Manifestations |
| D009461 | Neurologic Manifestations |
| D009422 | Nervous System Diseases |
| D010335 | Pathologic Processes |
| D013568 | Pathological Conditions, Signs and Symptoms |
| D012816 | Signs and Symptoms |