Treating CTDs Related Fibrotic ILDs by Immunosuppressants: “Facts and Faults”

• Published in: Lung Vol. 192; no. 2; pp. 221 - 223
• Main Authors: Papiris, Spyros A., Kagouridis, Konstantinos, Papadaki, Georgia, Kolilekas, Likurgos, Manali, Effrosyni D.
• Format: Journal Article
• Language: English
• Published: New York Springer US 01.04.2014; Springer; Springer Nature B.V
• Subjects: Anti-Inflammatory Agents - therapeutic use; Care and treatment; Complications and side effects; Cyclophosphamide - therapeutic use; Dosage and administration; Drug therapy; Female; Humans; Immunosuppressive agents; Lung Diseases, Interstitial - drug therapy; Lung Diseases, Interstitial - etiology; Male; Medicine; Medicine & Public Health; Mycophenolic Acid - analogs & derivatives; Patient outcomes; Pneumology/Respiratory System; Pneumonia; Pulmonary fibrosis; Risk factors; Scleroderma (Disease); Scleroderma, Systemic - complications; Systemic scleroderma; Greece; United States; Immunosuppressants; Cyclophosphamide; Pulmonary fibrosis; Treatment; Fibrotic interstitial lung diseases; Mycophenolate mofetil; Scleroderma
• ISSN: 0341-2040; 1432-1750
• DOI: 10.1007/s00408-013-9532-y

Fibrotic interstitial lung diseases (ILDs) are commonly encountered in scleroderma where they significantly influence prognosis. The mainstay of treatment in idiopathic fibrotic ILDs for the past 30 years was based on the combined administration of prednisone and cyclophosphamide (CYC) or prednisone, azathioprine plus N -acetyl cysteine, recently proved ineffective and harmful. Rheumatologists also despite “facts” showing that CYC treatment has no beneficial impact on fibrotic ILDs in scleroderma continue to commit the same, in a manner of speaking, “faults” by “treating their fibrotic ILDs by immunosuppressants.” In this issue of the journal, Panopoulos et al. (Lung, 191, 483–489, 2013 ) recognizing the minimal effect of CYC on fibrotic ILDs in scleroderma patients and the increased use in clinical practice of mycophenolate mofetil (MMF) as an alternative, report that MMF use to replace CYC in this setting is not supported, confirming that restoration of purely fibrotic damage in the lungs remains one of the most challenging fields in medicine.