Interstitial Lung Disease Associated With Collagen-Vascular Disease Treatment & Management
- Author: Isabel F Pedraza, MD; Chief Editor: Zab Mosenifar, MD, FACP, FCCP more...
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.
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, 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).
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.
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.
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|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.
|Anti-Ro/SSA and anti-La/SSB||-||-||-||+||-||-||-|
|Anti-U1-RNP and anti-UN-70 kd||-||-||-||-||-||-||+|
|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.|
|Radiologic Pattern||RA||SLE||SD||Secondary SS||PM/DM||AS||MCTD|
|Interstitial pneumonitis, fibrosis||UIP and NSIP patterns||+||LIP pattern||±||+||Upper apical fibrosis||+|
|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||+||-||-|
|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.|