Nonspecific Interstitial Pneumonia/Fibrosis Completely Recovered by Adding Cyclophosphamide to Corticosteroids.

Abstract

Nonspecific interstitial pneumonia/fibrosis (NSIP) was first described by Katzenstein and Fiorelli in 1994 (Am J Surg Pathol 18: 136-147). Many reports have described that corticosteroids are effective for NSIP.Wedescribe a case of group II idiopathic NSIP in whomcyclophosphamide was administered since the initial response to corticosteroids had been insufficient. Lung biopsy was performed by video-assisted thoracoscopic surgery and NSIP was diagnosed pathologically, clinically and radiologically. Although the initial response to corticosteroids was insufficient, interstitial infiltrates on chest computed tomography improved dramatically after adding intravenous cyclophosphamide followed by oral cyclophosphamide. This case demonstrates that the addition of cyclophosphamide to corticosteroids might be a useful treatment for patients with NSIP. (Internal Medicine 41 : 867-870, 2002)

Reviewing primary Sjögren's syndrome: beyond the dryness - From pathophysiology to diagnosis and treatment

“…ILD is difficult to treat and results in an increase of dry cough and dyspnea, leading to decreased quality of life. ILD is usually treated with glucocorticoids but other immunosuppressive drugs are also available, such as azathioprine, mycophenolate mofetil, cyclophosphamide and cyclosporine 64, 65. Furthermore, renal involvement is common and includes a wide spectrum of manifestations, of which interstitial nephritis is the most prevalent 66, 67.…”

Interstitial lung disease guideline

“…If there is improvement as judged by physiological and/or radiological criteria, this could be reduced to 10 mg/day or 20 mg/alternate days. A failure to respond or deterioration during this steroid trial should prompt an evaluation of immunosuppressant treatment such as cyclophosphamide,521 azathioprine,522 D-penicillamine522 or methotrexate, although there are only limited supportive data for any of these regimes. Cyclosporin A has been useful as a steroid-sparing agent when other agents have failed or disease has progressed despite corticosteroids and immunosuppressants 522 523.…”

The lung in rheumatoid arthritis

“…Glucocorticoid therapy is the treatment of choice with variable subjective and objective improvement in the treatment of RA-ILD [14,15,21]. Other drugs reported to be beneficial include cyclophosphamide, azathioprine, hydroxychloroquine, D-penicillamine, and cyclosporine [50][51][52]. The impact of newer therapies for RA (e.g., anti-TNF-alpha regimens), which decrease inflammation and may interfere with fibrosis, is still unknown [53].…”