Management of Systemic-Sclerosis-Associated Interstitial Lung Disease

Abstract

Although scleroderma-associated interstitial lung disease (SSc-ILD) is a significant contributor to both morbidity and mortality, its pathogenesis is largely unclear. Pulmonary function tests (PFTs) and high resolution CT (HRCT) scanning continue to be the most effective tools to screen for lung involvement and to monitor for disease progression. More research and better biomarkers are needed to identify patients most at risk for developing SSc-ILD as well as to recognize which of these patients will progress to more severe disease. While immunosuppression remains the mainstay of treatment, anti-fibrotic agents may offer new avenues of treatment for patients with SSc-ILD in the future.

Rituximab therapy is more effective than cyclophosphamide therapy for Japanese patients with anti‐topoisomerase I‐positive systemic sclerosis‐associated interstitial lung disease

“…Furthermore, given the risk of irreversible amenorrhea, it is difficult to treat CYC in young women who want to become pregnant . For the above reasons, a recent survey of SSc experts revealed a lack of consensus on treatment decisions of SSc‐ILD for both induction as well as maintenance therapy . Taken together, as there is not yet enough treatment for SSc‐ILD, development of an effective and safe treatment is desired.…”

Enfermedad pulmonar intersticial en pacientes con esclerosis sistémica. Revisión narrativa de la literatura

“…Por este motivo, la enfermedad pulmonar con sus dos manifestaciones: hipertensión pulmonar (HTP) y enfermedad pulmonar intersticial difusa (EPID), pasó a ser la principal causa de mortalidad, dando cuenta del 30 y 35% respectivamente (2). Esto se evidenció en la cohorte de Pittsburgh, Seguimiento a largo plazo, y se vio un cambio en la mortalidad por crisis renal que pasó de 42 % a 6 %; al contrario, la enfermedad pulmonar aumentó de 6 a 33% (3), convirtiéndose en una prioridad en el tratamiento de los pacientes con ES.…”

Is computed tomography the ideal method for the identification and management of lung disease in systemic sclerosis?

“…Involvement of the respiratory system is quite common in the course of systemic sclerosis (SSc) and can affect all its components, including the parenchyma, vasculature, airways, pleura, and muscle tissue (1) . However, the forms that are the most common and have the greatest clinical repercussions are interstitial lung disease (ILD) and pulmonary arterial hypertension, which account for up to 90% and 60% of cases, respectively (1,2) .…”

“…However, the forms that are the most common and have the greatest clinical repercussions are interstitial lung disease (ILD) and pulmonary arterial hypertension, which account for up to 90% and 60% of cases, respectively (1,2) . The presence of a pulmonary lesion is a determining factor in the quality of life of patients with SSc and currently represents the main cause of SScrelated mortality, especially among patients diagnosed with ILD (1) . The onset of SSc-ILD is insidious, with subtle clinical symptoms, which explains why the disease is often diagnosed in its advanced stages, when extensive pulmonary fibrosis is already present.…”

“…Although honeycombing is an indicator of poor prognosis (2) , the main marker of unfavorable evolution is the extent of the disease, which, when combined with the deterioration of lung function, best defines the prognosis (1) . The absence of findings consistent with fibrosis in the initial HRCT of patients with SSc is highly predictive of a "fibrosis-free" follow-up HRCT, whereas a finding of more than 20% fibrosis in the initial exam is associated with a high annual fibrosis progression rate, a decline in lung function, and the development of pulmonary arterial hypertension (3) .…”