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Interstitial Lung Disease Associated With Collagen-Vascular Disease Treatment & Management

  • Author: Isabel F Pedraza, MD; Chief Editor: Zab Mosenifar, MD, FACP, FCCP  more...
Updated: Sep 20, 2013

Approach Considerations

Patients presenting with an acute onset of dyspnea, hypoxia, and hypoxemia need hospital admission and close monitoring.

High-dose steroids have been associated with renal crisis in scleroderma and therefore are no longer recommended. In addition, although many types of CVD-related ILDs respond to immunosuppression, the decision to initiate treatment should be made after a careful assessment of the risks and benefits, and the likelihood of a response to therapy in that particular situation. Comorbidities such as DM, osteoporosis, and psychiatric disease should be carefully considered, as often more prolonged courses of steroids are indicated. Further, the addition of steroid-sparing medications can be considered depending on the CVD being treated. Lastly, if a patient shows progressive deterioration in lung function or shows no slowing in this decline, discontinuation of the immunosuppressive medications should be considered.

Most patients can be treated in an outpatient setting. Chest radiography, the 6-minute walk test, arterial blood gas determinations (ie, arterial oxygen tension [PaO2]), and pulmonary function tests (PFTs)—especially forced vital capacity (FVC) and diffusion capacity of the lung for carbon monoxide (DLCO)—are monitored after therapy is started. Monthly follow-up is recommended. Transfer to a tertiary care center is indicated if the diagnosis is in doubt or if treatment is ineffective.

Subspecialty consultations (eg, a pulmonologist and a rheumatologist) are required for proper diagnosis and management of collagen-vascular disease (CVD) associated with interstitial lung disease (ILD). Failure to refer the patient for such consultations is a potential pitfall. CVDs frequently involve the gastrointestinal (GI) tract; thus, special considerations (nutritional support and GI evaluation) may be needed.

Patients should be educated about the natural history, progression, and treatment of the disease. Before any immunosuppressive medication is started, the potential adverse effects, the duration of therapy, and the chances of success should be discussed with the patient.


Pharmacologic Therapy

CVDs are usually treated with immunosuppressive drugs, which are highly toxic and can cause drug-mediated lung disease. The decision to start therapy depends on the patient’s age, the severity of the disease, and the rapidity with which the disease is progressing. Drugs used to treat CVDs include corticosteroids, azathioprine, cyclosporine, cyclophosphamide,[62] and methotrexate.

In general, corticosteroids are not very useful in the treatment of scleroderma (SD). ILD caused by rheumatoid arthritis (RA) is treated with a corticosteroid or with a combination of a corticosteroid and azathioprine or cyclophosphamide. However, methotrexate is being used increasingly often in the management of ILD induced by RA or systemic lupus erythematosus (SLE).[63]

The role of biologic inhibitors (eg, tumor necrosis factor–α [TNF-α] inhibitors) in the treatment of RA-related ILD is currently uncertain. Hagiwara et al described a case in which an acute exacerbation of preexisting ILD occurred after the administration of etanercept for RA.[29]

Acute lupus pneumonitis should be treated with oxygen, broad-spectrum antibiotics, and corticosteroids.

Secondary pulmonary hypertension in patients with CVD is usually resistant to treatment and is associated with a poor prognosis. Patients should be referred to a center where experts in the evaluation of pulmonary hypertension are available.

BOOP has a good prognosis. It is usually treated with corticosteroids (prednisone 1 mg/kg/day). PM/DM-related ILD is usually fatal; case reports have described patients who were treated with a combination of corticosteroids and cyclophosphamide, with variable results.

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.

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[46] +
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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