Treatment and prognosis of interstitial lung disease in systemic sclerosis (scleroderma)
- John Varga, MD
John Varga, MD
- John and Nancy Hughes Professor of Medicine
- Northwestern University Feinberg School of Medicine
- Andrew M Tager, MD
Andrew M Tager, MD
- Associate Professor of Medicine
- Division of Pulmonary and Critical Care Medicine and Center for Im
- Section Editors
- Talmadge E King, Jr, MD
Talmadge E King, Jr, MD
- Editor-in-Chief — Pulmonary, Critical Care, and Sleep Medicine
- Section Editor — Interstitial Lung Disease
- Dean, School of Medicine
- Vice Chancellor, Medical Affairs
- University of California San Francisco
- John S Axford, DSc, MD, FRCP, FRCPCH
John S Axford, DSc, MD, FRCP, FRCPCH
- Section Editor — Scleroderma
- Emeritus Professor of Rheumatology
- St George's University of London
Interstitial lung disease (ILD) is a frequent complication of systemic sclerosis (SSc) that is often progressive and has a poor prognosis [1-4]. In a retrospective study of 619 patients with SSc, 40 percent of patients had a restrictive ventilatory defect (suggesting ILD) either alone or in combination with pulmonary arterial hypertension .
The treatment and prognosis of SSc-ILD will be reviewed here. The clinical presentation and diagnosis of SSc lung disease, the treatment of SSc in general, and the treatment of SSc-associated pulmonary arterial hypertension are discussed separately. (See "Clinical manifestations, evaluation, and diagnosis of interstitial lung disease in systemic sclerosis (scleroderma)" and "Overview of pulmonary complications of systemic sclerosis (scleroderma)" and "Overview of the treatment and prognosis of systemic sclerosis (scleroderma) in adults" and "Pulmonary arterial hypertension in systemic sclerosis (scleroderma): Definition, classification, risk factors, screening, and prognosis".)
TYPES OF INTERSTITIAL LUNG DISEASE
The term interstitial lung disease (ILD) is broadly used to describe a heterogeneous group of disorders that are classified together because of similar clinical, radiographic, physiologic, or pathologic manifestations. In this topic review, we refer broadly to systemic sclerosis (SSc)-ILD and do not distinguish among the histopathologic subtypes.
In the vast majority of patients with SSc-ILD, the lung injury is characterized by a pattern termed nonspecific interstitial pneumonia (NSIP) . Histopathologically, NSIP is characterized by varying degrees of pulmonary inflammation and fibrosis, with some forms being primarily inflammatory (cellular NSIP) and others primarily fibrotic (fibrotic NSIP). Most investigators believe that cellular NSIP is the early stage of fibrotic NSIP. Although NSIP may have significant fibrosis, it is usually of uniform temporality. Fibroblastic foci and honeycombing, if present, are rare. (See "Idiopathic interstitial pneumonias: Clinical manifestations and pathology", section on 'Nonspecific interstitial pneumonia'.)
A minority of patients with SSc-ILD have the histopathologic pattern of usual interstitial pneumonia (UIP), the pattern that is also associated with idiopathic pulmonary fibrosis (IPF). This pattern is characterized by a non-uniform distribution of alternating zones of dense fibrosis, fibroblast foci, scant inflammation, normal lung, and honeycomb change.
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- TYPES OF INTERSTITIAL LUNG DISEASE
- INDICATIONS FOR TREATMENT
- INITIAL THERAPY
- - Intravenous cyclophosphamide
- - Oral cyclophosphamide
- - Duration
- - Adverse effects and contraindications
- Adjuvant glucocorticoids
- Alternative initial therapy
- - Mycophenolate mofetil
- - Azathioprine
- SUBSEQUENT MAINTENANCE THERAPY
- REFRACTORY DISEASE
- Lung transplantation
- MONITORING RESPONSE TO THERAPY
- SUPPORTIVE CARE
- INVESTIGATIONAL APPROACHES
- Hematopoietic stem cell transplantation
- AGENTS WITHOUT CLEAR BENEFIT
- SUMMARY AND RECOMMENDATIONS