title: Management of connective tissue diseases associated interstitial lung disease: a review of the published literature.
abstract: OBJECTIVE
  Interstitial lung disease (ILD), though a common and often a severe manifestation of many connective tissue diseases (CTD), is challenging to manage because of its variable presentation and the relative lack of guidelines to assist the clinician. In this review, we discuss the approach to diagnosis, treatment, and monitoring patients with CTD-associated ILD, with a focus on systemic sclerosis (SSc), rheumatoid arthritis (RA), and idiopathic inflammatory myopathy (IIM).
  
  
  RESULTS
  High-resolution computed tomography scan and pulmonary function testing can be reliably used to diagnose ILD and monitor progression, and often to determine its likely histologic subtype and severity. In SSc-ILD, randomized controlled trials show ILD stabilization with cyclophosphamide treatment; preliminary data from another randomized controlled trial demonstrates similar findings with mycophenolate. There are no robust clinical trials supporting specific treatments for RA-ILD or IIM-ILD, but rituximab in RA-ILD, and cyclophosphamide, mycophenolate and calcineurin inhibitors in IIM-ILD show promise.
  
  
  CONCLUSIONS
  Though ILD contributes substantially to morbidity and mortality in patients with CTD, there are minimal data to guide its management except in SSc-ILD.
authors.affiliation: aDepartment of RheumatologybDepartment of Radiology, University of Michigan Health System, Ann Arbor, Michigan, USA.
authors.forename: Beth
authors.initials: B
authors.lastname: Wallace
authors.forename: Dharshan
authors.initials: D
authors.lastname: Vummidi
authors.forename: Dinesh
authors.initials: D
authors.lastname: Khanna
mesh_terms: D003240:Connective Tissue Diseases / Q000150:complications / Q000175:diagnosis / Q000628:therapy; D003952:Diagnostic Imaging / Q000379:methods*; D019468:Disease Management; D018450:Disease Progression; D006801:Humans; D007165:Immunosuppression Therapy / Q000379:methods*; D017563:Lung Diseases, Interstitial / Q000175:diagnosis / Q000209:etiology / Q000628:therapy