Therapeutic options for systemic sclerosis related interstitial lung diseases.

Interstitial lung disease (ILD) is a common manifestation of systemic sclerosis (SSc) mainly encountered in patients with diffuse disease. Unlike idiopathic interstitial pneumonias (IIP), SSc associated ILD corresponds to non-specific interstitial pneumonia (NSIP) in most cases, whereas usual interstitial pneumonia (UIP) is encountered less frequently. This explains the better prognosis of SSc associated ILD compared to IIP. However, severe restrictive lung disease represents one of the two main causes of disease-related death in SSc patients. The treatment of SSc associated ILD is not very well established. Anti-fibrosing treatments have failed to demonstrate any benefit and cyclophosphamide, which has been used in the treatment of this condition for about 15 years, has recently been evaluated in two prospective randomised studies which showed a significant but modest effect on respiratory function. Since none of the patients included in retrospective or prospective studies were selected on the basis of progression of ILD, and since only a minority of SSc patients develop severe ILD, further studies should focus on the subgroup of SSc patients with worsening ILD. A subgroup of patients with rapidly progressive ILD might benefit from pulsed intravenous cyclophosphamide combined with prednisone 15 mg daily but this remains to be confirmed.

Mouthon L ，Bérezné A ，Guillevin L ，Valeyre D ... -  《-》

Interstitial lung disease associated with systemic sclerosis: what is the evidence for efficacy of cyclophosphamide?

Bérezné A ，Valeyre D ，Ranque B ，Guillevin L ，Mouthon L ... -  《ANNALS OF THE NEW YORK ACADEMY OF SCIENCES》

[Interstitial lung disease in systemic sclerosis].

Mouthon L ，Berezné A ，Brauner M ，Kambouchner M ，Guillevin L ，Valeyre D ... -  《REVUE DES MALADIES RESPIRATOIRES》

Interstitial lung disease in systemic sclerosis.

Interstitial lung disease (ILD) is a common manifestation of systemic sclerosis (SSc) and mainly encountered in patients with diffuse disease and/or anti-topoisomerase 1 antibodies. ILD develops in up to 75% of patients with SSc overall. However, SSc-ILD evolves to end-stage respiratory insufficiency in only a few patients. Initial pulmonary function tests (PFT) with measurement of carbon monoxide diffusing capacity, together with high-resolution computed tomography, allows for early diagnosis of SSc-ILD, before the occurrence of dyspnea. Unlike idiopathic ILD, SSc-ILD corresponds to non-specific interstitial pneumonia in most cases, whereas usual interstitial pneumonia is less frequently encountered. Therefore, the prognosis of SSc-ILD is better than that for idiopathic ILD. Nevertheless, ILD represents one of the two main causes of death in SSc patients. To detect SSc-ILD early, PFT must be repeated regularly, every 6 months to 1 year, depending on disease worsening. Conversely, broncho-alveolar lavage is not needed to evaluate disease activity in SSc-ILD but may be of help in diagnosing opportunistic infection. The treatment of SSc-ILD is not well established. Cyclophosphamide, which has been used for 20 years, has recently been evaluated in two prospective randomized studies that failed to demonstrate a major benefit for lung function. Open studies reported mycophenolate mofetil, azathioprine and rituximab as alternatives to cyclophosphamide. On failure of immunosuppressive agent treatment, lung transplantation can be proposed in the absence of other major organ involvement or severe gastro-esophageal reflux.

Bussone G ，Mouthon L 《-》

Review series: Aspects of interstitial lung disease: connective tissue disease-associated interstitial lung disease: how does it differ from IPF? How should the clinical approach differ?

de Lauretis A ，Veeraraghavan S ，Renzoni E 《-》