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Interstitial Lung Disease Associated With Collagen-Vascular Disease Medication

  • Author: Isabel F Pedraza, MD; Chief Editor: Zab Mosenifar, MD, FACP, FCCP  more...
Updated: Aug 12, 2016

Medication Summary

The goals of pharmacotherapy are to reduce morbidity and prevent complications. Drugs used to manage collagen-vascular disease (CVD) associated with interstitial lung disease (ILD) include corticosteroids and antineoplastic agents.[67]



Class Summary

Corticosteroids have anti-inflammatory properties and cause profound and varied metabolic effects. They modify the body’s immune response to diverse stimuli.

Prednisone (Deltasone, Rayos)


Prednisone is used as an immunosuppressant in the treatment of autoimmune disorders. It has anti-inflammatory properties and produces multiple glucocorticoid and mineralocorticoid effects. Prednisone therapy is best prescribed in consultation with a pulmonary disease specialist.

Prednisolone (FloPred, Millipred, Millipred DP, Prelone)


Elicits mild mineralocorticoid activity and moderate anti-inflammatory effects; controls or prevents inflammation by controlling rate of protein synthesis, suppressing migration of polymorphonuclear leukocytes (PMNs) and fibroblasts, reversing capillary permeability, and stabilizing lysosomes at cellular level.



Class Summary

These agents inhibit key factors involved in immune reactions.

Azathioprine (Imuran, Azasan)


Azathioprine is an imidazolyl derivative of 6-mercaptopurine (6-MP). Many of its biologic effects are similar to those of the parent compound. Both compounds are eliminated rapidly from blood and are oxidized or methylated in erythrocytes and liver. No azathioprine or 6-MP is detectable in the urine 8 hours after being taken.

Azathioprine antagonizes purine metabolism and inhibits synthesis of DNA, RNA, and proteins. The mechanism whereby it affects autoimmune diseases is unknown. It works primarily on T cells, suppressing hypersensitivities of the cell-mediated type and causing variable alterations in antibody production. Immunosuppressive, delayed hypersensitivity, and cellular cytotoxicity test results are suppressed to a greater degree than antibody responses. Azathioprine works very slowly; a 6- to 12-month trial may be required before effects are observed.

As many as 10% of patients may have idiosyncratic reactions that rule out the use of azathioprine. Do not allow the white blood cell (WBC) count to drop below 3000/µL or the lymphocyte count to drop below 1000/µL. The drug is available in tablet form for oral administration or in 100-mg vials for intravenous (IV) injection.

Cyclosporine (Gengraf, Neoral, Sandimmune)


Cyclosporine is a cyclic polypeptide that suppresses some humoral immunity and, to a greater extent, cell-mediated immune reactions. Suppresses mRNA expression of Th2 cytokines (interleukins 4 and 13) in peripheral blood mononuclear cells.


Antineoplastic Agents

Class Summary

Antineoplastic agents inhibit cell growth and proliferation.



Cyclophosphamide is primarily used for treating several types of cancer and autoimmune disorders. It is related to the nitrogen mustard group, and its mode of action involves alkylation and cross-linking of DNA. Cyclophosphamide is used for several different types of rheumatic disease, including systemic lupus erythematosus (SLE) and sometimes rheumatoid arthritis (RA).

Methotrexate (Trexall, Rheumatrex, Otrexup, Rasuvo)


Methotrexate is an antimetabolite used in the treatment of certain neoplastic diseases, severe psoriasis, and adult rheumatoid arthritis. It inhibits dihydrofolic acid reductase. Dihydrofolates must be reduced to tetrahydrofolates by this enzyme before they can be used as carriers of single-carbon groups in the synthesis of purine nucleotides and thymidylate. Methotrexate therefore interferes with DNA synthesis, repair, and cellular replication.

Contributor Information and Disclosures

Isabel F Pedraza, MD Director, Respiratory Intensive Care Unit, Faculty Physician, Department of Medicine, Division of Pulmonary/Critical Care Medicine, Women's Guild Lung Institute, Cedars-Sinai Medical Center

Disclosure: Nothing to disclose.


Daniel R Ouellette, MD, FCCP Associate Professor of Medicine, Wayne State University School of Medicine; Chair of the Clinical Competency Committee, Pulmonary and Critical Care Fellowship Program, Senior Staff and Attending Physician, Division of Pulmonary and Critical Care Medicine, Henry Ford Health System; Chair, Guideline Oversight Committee, American College of Chest Physicians

Daniel R Ouellette, MD, FCCP is a member of the following medical societies: American College of Chest Physicians, Society of Critical Care Medicine, American Thoracic Society

Disclosure: Nothing to disclose.

Arshad Ali, MD Attending Physician, Department of Pulmonary and Critical Care Medicine, Mercy General Hospital of Sacramento

Arshad Ali, MD is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, American Thoracic Society

Disclosure: Nothing to disclose.

Danilo A Enriquez, MD, FCCP Clinical Assistant Professor of Medicine, State University of New York Health Science Center at Brooklyn; Associate Program Director of Internal Medicine Residency Program, Interfaith Medical Center

Danilo A Enriquez, MD, FCCP is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, American Medical Association

Disclosure: Nothing to disclose.

Hina Arif, MD 

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Zab Mosenifar, MD, FACP, FCCP Geri and Richard Brawerman Chair in Pulmonary and Critical Care Medicine, Professor and Executive Vice Chairman, Department of Medicine, Medical Director, Women's Guild Lung Institute, Cedars Sinai Medical Center, University of California, Los Angeles, David Geffen School of Medicine

Zab Mosenifar, MD, FACP, FCCP is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, American Federation for Medical Research, American Thoracic Society

Disclosure: Nothing to disclose.


Om Prakash Sharma, MD, FRCP, FCCP, DTM&H Professor, Department of Medicine, Division of Pulmonary and Critical Care Medicine, University of Southern California Keck School of Medicine

Om Prakash Sharma, MD, FRCP, FCCP, DTM&H is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American College of Chest Physicians, American College of Physicians, American Federation for Medical Research, American Osler Society, American Thoracic Society, New York Academy of Medicine, and Royal Society of Medicine

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

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Pulmonary hypertension is complication of various collagen-vascular diseases. Lung biopsy specimen demonstrates severe interstitial fibrosis and medial fibrosis and smooth muscle hyperplasia of pulmonary arteriole, compatible with pulmonary hypertension.
Heliotrope rash in woman with dermatomyositis.
Gottron papules and nail-fold telangiectasia in patient with dermatomyositis.
Classic malar rash (butterfly rash) with distribution over cheeks and nasal bridge. Note that fixed erythema (sometimes associated with mild induration, as here) characteristically spares nasolabial folds.
High-resolution CT scan of advanced-stage pulmonary fibrosis demonstrating reticular opacities with honeycombing in predominantly subpleural distribution. This pattern can be present in rheumatoid arthritis–related interstitial lung disease, Sjögren syndrome, and scleroderma.
Ground-glass opacification (GGO) may correlate with active alveolitis and favorable response to therapy. GGO is among earliest features of rheumatoid arthritis–induced interstitial lung disease.
Patient with lymphocytic interstitial pneumonia.
Usual interstitial pneumonitis. Subpleural and paraseptal inflammation are present, with appearance of temporal heterogeneity. Patchy scarring of lung parenchyma and normal (or nearly normal) alveoli interspersed between fibrotic areas are hallmarks of this disease. In addition, lung architecture is completely destroyed. This pattern can be present in rheumatoid arthritis–induced interstitial lung disease and generally is associated with poor prognosis.
Table 1. Important Physical Findings in Collagen-Vascular Diseases
CVD Skin and Musculoskeletal System Lungs Heart Salivary Glands Eyes
RA* Subcutaneous nodules, digital ulcers, nail-fold infarcts Bibasilar Velcro crackles, signs of pulmonary hypertension, pleural effusion Pericarditis, myocarditis N/A N/A
SLE* Malar rash, alopecia, livedo reticularis, erythema, telangiectasia, capillary infarcts, polyarthritis Pleural effusion or rub, pneumonitis, cor pulmonale, diaphragmatic weakness Pericarditis, myocarditis, CAD N/A N/A
SD Thickening of skin of face, fingers, and hands; Raynaud phenomenon and ischemic changes of fingertips Cor pulmonale, inspiratory Velcro crackles at lung bases Restrictive pericardial disease, conduction defects, CHF N/A N/A
SS* Secondary SS can manifest similarly to RA and SLE Secondary SS can manifest similarly to RA and SLE N/A Xerostomia, parotid gland swelling Keratoconjunctivitis sicca
PM Proximal muscle weakness Respiratory muscle failure N/A N/A N/A
DM Heliotrope rash of eyelids, Gottron papules, Respiratory muscle failure N/A N/A N/A
AS Sacroiliitis Restriction in chest expansion, pulmonary apical fibrosis Aortic insufficiency N/A Anterior uveitis
* MCTD can manifest with the signs and symptoms of RA, SLE, or SS.

AS = ankylosing spondylitis; CAD = coronary artery disease; CHF = congestive heart failure; CVD = collagen-vascular disease; DM = dermatomyositis; PM = polymyositis; RA = rheumatoid arthritis; SD = scleroderma; SLE = systemic lupus erythematosus; SS = Sjögren syndrome.

Table 2. Autoantibodies in Collagen-Vascular Diseases
RF + + + + Rare - +
ANA + + + + Rare - +


ds-DNA - + - - - - -
Anticentromere - - + (limited) Rare Rare - -
Scl-70 - - + (diffuse)   Rare - -
Anti-Jo - - - Rare + (ILD) - -
ANCA Rare Rare - - - - -
Smith antibody - + - - - - -
Anti-Ro/SSA and anti-La/SSB - - - + - - -
Anti-U1-RNP and anti-UN-70 kd - - - - - - +
Anti-CCP + - - - - - -
ANA = antinuclear antibody; ANCA = antineutrophilic cytoplasmic antibody; AS = ankylosing spondylitis; DM = dermatomyositis; ds-DNA = double-stranded DNA antibody; ILD = interstitial lung disease; MCTD = mixed connective-tissue disease; PM = polymyositis; RA = rheumatoid arthritis; RF = rheumatoid factor; RNP = ribonucleoprotein; SD = scleroderma; SLE = systemic lupus erythematosus; SS = Sjögren syndrome; CCP = cyclic citrullinated peptide.
Table 3. Radiographic Patterns of Collagen-Vascular Diseases
Radiologic Pattern RA SLE SD Secondary SS PM/DM AS MCTD
Pleural effusion + + + ± - - +
Interstitial pneumonitis, fibrosis UIP and NSIP patterns + LIP pattern ± + Upper apical fibrosis[49] +
BOOP ± + + + + ± ±
Pulmonary nodules Rheumatoid pulmonary nodules; uncommon, may be 1-5 mm, single or multiple, may cavitate - - Follicular lymphoid hyperplasia or lymphoma can present as lung nodules + - -
Bronchiectasis + + + + + + +
Caplan syndrome Coal worker’s pneumoconiosis, rheumatoid nodules - - - - - ±
Diffuse pulmonary hemorrhage - + - - - - ±
Shrinking lung syndrome - Loss of lung volume at bases with no parenchymal pathology - - - - ±
Diaphragmatic dysfunction - + - May be present + - ±
Cysts, honeycombing 10% of patients have subpleural honeycombing; compared with IPF, it is more anterior and involves upper lobes Uncommon + Present, especially in LIP + Upper-lobe cyst may become infected with Aspergillus species -
GGO Present, especially in NSIP + + + + - ±
AS = ankylosing spondylitis; BOOP = bronchiolitis obliterans organizing pneumonia; DM = dermatomyositis; GGO = ground-glass opacification; IPF = idiopathic pulmonary fibrosis; LIP = lymphoid interstitial pneumonia; MCTD = mixed connective-tissue disease; NSIP = nonspecific interstitial pneumonia; PM = polymyositis; RA = rheumatoid arthritis; SD = scleroderma; SLE = systemic lupus erythematosus; SS = Sjögren syndrome; UIP = usual interstitial pneumonia.
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