Respiratory Disorders Associated With Collagen Vascular Disease
Paul C. Stillwell, MD, FAAP, and Robin R. Deterding, MD
Introduction/Etiology/Epidemiology
•Collagen vascular diseases (CVDs), also known as connective tissue diseases or rheumatic diseases, are a diverse group of multisystem diseases caused by autoimmune antibodies.
•Table 89-1 shows the relative frequency of the individual CVDs in children. Table 89-2 shows the type of lung disease and the relative frequency for each CVD. This group includes
—Juvenile idiopathic arthritis (JIA)
▪Of the 8 subtypes of JIA, pulmonary involvement most often occurs with systemic-onset JIA and rheumatoid factor–positive polyarticular arthritis.
—Systemic lupus erythematosis
—Juvenile systemic sclerosis
AA, African American; ANA, antinuclear antibody; anti-dsDNA, anti–double-stranded DNA antibody; anti-Scl-70, anti-topoisomerase 1 antibody; JDM, juvenile dermatomyositis; JIA, juvenile idiopathic arthritis; JSS, juvenile systemic sclerosis; RF, rheumatoid factor; SLE, systemic lupus erythematosus.
—Juvenile dermatomyositis
—Mixed connective tissue disease
—Juvenile ankylosing spondylitis
—Sjogren syndrome
•The most commonly occurring CVDs have the lowest incidence of pulmonary involvement; the less commonly occurring CVDs have a higher risk of pulmonary involvement.
•There is considerable overlap between the lung involvement with CVD, pulmonary vasculitis, and granulomatous lung disease (see Chapter 90, Vasculitis-Related Respiratory Disorders, and Chapter 91, Granulomatous Respiratory Disorders).
—Although not explicitly a rheumatologic disease, pulmonary capillaritis is a neutrophilic vasculitis in the lung that results in life-threatening pulmonary hemorrhage.
—These patients may develop a CVD over time and are frequently treated with many of the same agents used for CVD.
•In addition to the pulmonary involvement by the primary CVD, treatment of the CVD often renders the patient immunocompromised; therefore, opportunistic infections may be a cause of the respiratory disease.
•Some of the therapies for the CVD can be responsible for direct lung injury, such as methotrexate, and some of the new biological treatments, such as rituximab or rapamycin (sirolimus), can cause clinically significant immunosuppression.
Clinical Features
•Pulmonary symptoms may be the initial manifestation of the CVD.
•The symptoms of pulmonary involvement can be subtle and insidious.
—Dyspnea with activity
—Dry cough
—Fatigue
•With alveolar hemorrhage, the presentation can be dramatic.
—Severe dyspnea
—Hemoptysis
•Crackles may be heard with interstitial lung disease.
•Dullness on percussion may be noted if there is a pleural effusion.
•The pulmonic component of the second heart sound may be increased when pulmonary hypertension is present.
•Wheezing may be evident with bronchiolitis obliterans, which is a rare disease process that can co-travel with CVD.
Diagnostic Considerations
•The diagnosis of the CVD is usually established with the assistance of a rheumatologist.
•The type of pulmonary disease is discerned by knowing the CVD type and what pulmonary diseases might be associated with that particular CVD.
•Clarification of pulmonary involvement is aided by findings at chest radiography, chest computed tomography, and, often, flexible bronchoscopy.
•Lung biopsy is usually required for the specific diagnosis of interstitial lung disease or pulmonary capillaritis.
•Bronchoscopy with bronchoalveolar lavage helps determine whether the pulmonary issue is an opportunistic infection, alveolar hemorrhage, or pulmonary alveolar proteinosis.
Treatment
•Treatment of the underlying CVD is usually treatment of the pulmonary component, as well, and specific treatment varies considerably, depending on the specific CVD.
•Common therapies include glucocorticoids, cyclophosphamide, methotrexate, and, more recently, biological agents such as rituximab (monoclonal antibody against CD20 protein) and anakinra (interleukin-1 receptor antagonist).
•Infectious complications often require intravenous antibiotics or antivirals.
•The short-term prognosis depends on the response of the primary CVD to initial therapy but is commonly favorable.
•Alveolar hemorrhage can be fatal, despite appropriate therapy.
•Recurrence of the pulmonary process is common with flares of the CVD.
•Progression to end-stage fibrosis may occur when ILD or pulmonary alveolar proteinosis with systemic JIA is present.
•If pulmonary hypertension is present, the prognosis is guarded.
When to Refer
•Pulmonary involvement with the CVDs requires multidisciplinary team involvement from pulmonology, rheumatology, infectious diseases, immunology, and, often, critical care medicine. Referral for evaluation and establishment of a follow-up plan should happen at the earliest sign of pulmonary disease.
Resource for Families
•Collagen Vascular Lung Disease (American Thoracic Society). www.thoracic.org/patients/patient-resources/breathing-in-america/resources/chapter-6-collagen-vascular-lung-disease.pdf
Clinical Pearl
•Symptoms of pulmonary disease may be the first indication of the presence of a CVD or may develop gradually over time, long after the diagnosis is established. All patients with CVD should be screened and followed up for pulmonary involvement, with a minimum of oxygen saturation evaluations and pulmonary function testing for anyone with pulmonary symptoms.
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