The collagen vascular diseases (Table 11–1)
are a heterogeneous group of idiopathic, inflammatory, systemic
diseases. Common to these diseases is an aberrant immunological
response resulting in end-organ damage. Pulmonary involvement results
in significantly increased morbidity and mortality independent of
that associated with the underlying connective tissue disease. Therefore, physicians
must be aware of the pulmonary manifestations of the connective
tissue diseases. In doing so, appropriate therapy and surveillance
may be instituted to limit or ameliorate progressive dysfunction.
Collagen Vascular Diseases. |Favorite Table|Download (.pdf)
Collagen Vascular Diseases.
|Rheumatoid arthritis (RA)|
|Systemic lupus erythematosus (SLE)|
|Systemic sclerosis (SSc)|
|Mixed connective tissue disease (MCTD)|
|Systemic vasculitides (Wegener’s granulomatosis,
Pulmonary abnormalities occur in the airways, lung parenchyma,
pulmonary vasculature, or pleura. Dysfunction may be either a direct
effect of the underlying disease process or a secondary complication
due to treatment toxicities and opportunistic infection. Although
infectious complications are numerous, this chapter will focus solely
on the noninfectious aspects of lung involvement of the connective
tissue diseases. Specifically, the focus will be on the common pulmonary manifestations
of rheumatoid arthritis (RA), systemic lupus erythematosus (SLE),
systemic sclerosis (SSc), and polymyositis/dermatomyositis
(PM/DM). Pulmonary manifestations of less common diseases
such as Sjögren’s syndrome and mixed connective
tissue disease will be highlighted briefly. The pulmonary vasculitides
are discussed in Chapter 20: Vasculitis & the Diffuse Alveolar
- • Elevated rheumatoid factor.
- • Symmetric, small joint arthralgias with stiffness
that may progress to joint deformities.
- • Dyspnea with or without a productive cough.
Rheumatoid arthritis is a systemic inflammatory disease predominantly
affecting the synovial membranes of the diarthrodial joints. The
disease affects all ethnicities, with an increasing prevalence between
the fourth and sixth decades. In North America, the annual incidence
is estimated to be 0.3–1.5%. The prevalence in
women is 2.5 times higher than in men. However, pulmonary manifestations
(Table 11–2) are more common in men, with a 3:1
ratio, and in those who develop disease later in life.
Pulmonary Manifestations in Rheumatoid Arthritis. |Favorite Table|Download (.pdf)
Pulmonary Manifestations in Rheumatoid Arthritis.
|Airflow limitation (obstruction)|
|Bronchiolitis obliterans (constrictive)|
|Empyema (aseptic or septic)|
|Pneumothorax secondary to a ruptured necrobiotic nodule|
|Rheumatoid nodules (necrobiotic nodules)|
|Interstitial lung disease|
|Interstitial pulmonary fibrosis|
|Bronchiolitis obliterans organizing pneumonia|
|Apical fibrobullous disease|
|Drug-induced lung disease|
|Thoracic cage immobility|
|Pulmonary vascular disease|
|Pulmonary artery hypertension|
|Reactivation of tuberculosis|
Rheumatoid arthritis appears to be a disease in which there is
an aberrant immunological ...