Les pneumopathies infiltrantes diffuses de la sclérodermie systémique surviennent préférentiellement au cours des formes diffuses de la maladie.
Les pneumopathies infiltrantes diffuses de la ...sclérodermie systémique doivent être dépistées systématiquement par la réalisation d’une tomodensitométrie thoracique haute résolution et d’épreuves fonctionnelles respiratoires avec mesure de la capacité de transfert du monoxyde de carbone.
Ce sont habituellement des pneumopathies interstitielles non spécifiques, ce qui contribue à expliquer que leur pronostic est meilleur que celui de la fibrose pulmonaire idiopathique.
Cependant, les pneumopathies infiltrantes diffuses constituent aujourd’hui une des 2 premières causes de décès au cours de la sclérodermie systémique.
Le traitement des pneumopathies infiltrantes diffuses de la sclérodermie systémique n’est pas encore bien codifié. Le cyclophosphamide exerce un effet bénéfique significatif mais très modéré sur la fonction respiratoire.
Les patients avec une pneumopathie infiltrante diffuse rapidement progressive pourraient être traités par l’association de cyclophosphamide intraveineux à une corticothérapie par 15 mg/j de prednisone.
Interstitial lung diseases (ILD) associated with systemic sclerosis (SSc) are mainly encountered in patients with diffuse disease, although they may also be seen in patients with limited cutaneous SSc.
ILD screening must be performed regularly, with high-resolution computed tomography and pulmonary function tests (TLCO). Up to 75% of patients with diffuse SSc develop a form of ILD.
ILD remains stable in most patients and does not worsen.
The nonspecific nature of SSc-associated ILD makes it different from idiopathic ILD and helps to explain its better prognosis.
Nonetheless, ILD is one of the two leading causes of death in SSc patients.
Treatment of SSc-associated
ILD is not yet well codified.
Antifibrotic treatments have not proved beneficial, and the efficacy of cyclophosphamide, which has been used to treat this condition for 15 years, has been shown to be very limited against SSc-associated ILD.
A subgroup of patients with rapidly progressive ILD might benefit from intravenous cyclophosphamide pulses in association with 15 mg/d prednisone.
Objective
An algorithm for the detection of pulmonary arterial hypertension (PAH), based on the presence of dyspnea and the findings of Doppler echocardiographic evaluation of the velocity of ...tricuspid regurgitation (VTR) and right‐sided heart catheterization (RHC), which was applied in a large multicenter systemic sclerosis (SSc) population, estimated the prevalence of PAH to be 7.85%. The aim of this observational study was to investigate the incidence of PAH and pulmonary hypertension (PH) during a 3‐year followup of patients from the same cohort (the ItinérAIR‐Sclérodermie Study).
Methods
Patients with SSc and without evidence of PAH underwent evaluation for dyspnea and VTR at study entry and during subsequent visits. Patients in whom PAH was suspected because of a VTR of 2.8–3.0 meters/second and unexplained dyspnea or a VTR of >3.0 meters/second underwent RHC to confirm the diagnosis.
Results
A total of 384 patients were followed up for a mean ± SD of 41.03 ± 5.66 months (median 40.92 months). At baseline, 86.7% of the patients were women, and the mean ± SD age of the patients was 53.1 ± 12.0 years. The mean ± SD duration of SSc at study entry was 8.7 ± 7.6 years. After RHC, PAH was diagnosed in 8 patients, postcapillary PH in 8 patients, and PH associated with severe pulmonary fibrosis in 2 patients. The incidence of PAH was estimated to be 0.61 cases per 100 patient‐years. Two patients who exhibited a mean pulmonary artery pressure of 20–25 mm Hg at baseline subsequently developed PAH.
Conclusion
The estimated incidence of PAH among patients with SSc was 0.61 cases per 100 patient‐years. The high incidence of postcapillary PH highlights the value of RHC in investigating suspected PAH.
Les pneumopathies infiltrantes diffuses (PID) des connectivites s’opposent par certains points aux PID idiopathiques, mais partagent avec elles de nombreux aspects. Elles surviennent habituellement ...chez des patients ayant une connectivite connue ou de diagnostic simultané. Elles doivent être distinguées des pneumopathies d’autres mécanismes, notamment les infections opportunistes, les pneumopathies médicamenteuses et le poumon cardiaque. L’incidence des PID varie en fonction des connectivites. Les PID sont plus fréquentes au cours de la sclérodermie systémique et des myosites. À la différence de ce qui est observé dans les PID idiopathiques, leur substratum histopathologique le plus fréquent est la pneumopathie interstitielle non spécifique, alors que la pneumopathie interstitielle commune est plus rare. D’autres atteintes, comme la pneumopathie organisée, la pneumopathie interstitielle lymphoïde, un dommage alvéolaire diffus ou une hémorragie alvéolaire peuvent être observées. Les PID des connectivites doivent être recherchées systématiquement et leur retentissement doit être exploré par des explorations fonctionnelles respiratoires complètes et une estimation échocardiographique de la pression artérielle pulmonaire. Le pronostic des PID des connectivites est meilleur que celui des PID idiopathiques. Le traitement varie en fonction du type de connectivite et du degré d’évolutivité de la PID. Il est souvent efficace et repose sur les corticoïdes et/ou les immunosuppresseurs. La réhabilitation respiratoire et la transplantation pulmonaire peuvent être indiqués, le cas échéant.
Interstitial lung diseases (ILD) associated with connective tissue disorders differ from idiopathic ILD in several aspects, although most of them are comparable. In most patients, ILD occurs during the course, or at the time of diagnosis of connective tissue disease. Opportunistic pulmonary infections, together with adverse effects of treatment should always be discussed. The prevalence of ILD varies among the different connective tissue disorders. Thus, ILD is frequently encountered in patients with systemic sclerosis and to a lesser degree in patients with myositis. As compared to idiopathic ILD, histopathological aspects of ILD associated with connective tissue diseases are more frequently those of non-specific interstitial pneumonia, whereas usual interstitial pneumonia is rare. Other ILD, such as organized pneumonia, interstitial lymphoid pneumonia, diffuse alveolar damage and alveolar hemorrhage are occasionnaly encountered. ILD must be detected early in the course of collagen disorders by performing computed tomodensitometry and pulmonary function tests. The prognosis of connective tissue associated ILD is better than that of idiopathic ILD. The treatment requires corticosteroids and/or immunosuppressants, depending on the nature of the associated connective tissue disease and ILD progression.
Interstitial lung diseases (ILD) associated with connective tissue disorders differ from idiopathic ILD in several aspects, although most of them are comparable. In most patients, ILD occurs during ...the course, or at the time of diagnosis of connective tissue disease. Opportunistic pulmonary infections, together with adverse effects of treatment should always be discussed. The prevalence of ILD varies among the different connective tissue disorders. Thus, ILD is frequently encountered in patients with systemic sclerosis and to a lesser degree in patients with myositis. As compared to idiopathic ILD, histopathological aspects of ILD associated with connective tissue diseases are more frequently those of non-specific interstitial pneumonia, whereas usual interstitial pneumonia is rare. Other ILD, such as organized pneumonia, interstitial lymphoid pneumonia, diffuse alveolar damage and alveolar hemorrhage are occasionally encountered. ILD must be detected early in the course of collagen disorders by performing computed tomodensitometry and pulmonary function tests. The prognosis of connective tissue associated ILD is better than that of idiopathic ILD. The treatment requires corticosteroids and/or immunosuppressants, depending on the nature of the associated connective tissue disease and ILD progression.
