Pulmonary Complications of Collagen Vascular Disease

Abstract

Chronic interstitial lung disease complicates collagen vascular disorders in more than 5% of cases and is an important cause of morbidity and mortality in this patient population. An alveolitis characterizes the early phases of the lung lesion; perpetuation of this inflammatory process may result in irreversible pulmonary fibrosis and respiratory failure. In this chapter, we review the role of conventional studies (such as chest radiographs and pulmonary function tests) and newer diagnostic modalities (such as bronchoalveolar lavage, gallium scanning, and thin-section computed tomographic scanning) to identify patients with active alveolitis who are at risk for functional deterioration. While optimal treatment is controversial, the role of corticosteroids, immunosuppressive/cytotoxic, and antifibrotic agents is discussed.

Histopathology of Lung Disease in the Connective Tissue Diseases

“…44 Acute and organizing diffuse alveolar damage, with fibrin microthrombi, hemorrhage, and hyaline membranes in various stages of fibroblastic organization, is the most frequently reported manifestation of lupus lung disease. 43,[45][46][47] OP also seems to be a common finding among studies of lupus pneumonitis. [48][49][50] Finally, DAH is a potentially fatal acute complication of SLE, possibly as a result of capillary immune complex deposition, and can be seen as fresh hemorrhage or aggregates of hemosiderin laden (Fig.…”

Idiopathic Pulmonary Fibrosis: Diagnosis and Clinical Manifestations

“…While the clinical history helps, it may be misleading. CTDs can also mimic IPF, both clinically and radiologically 78,84–86. Elimination of specific symptoms and detection of autoantibodies can distinguish CTD from IPF.…”

“…CTDs can also mimic IPF, both clinically and radiologically. 78,84-86 Elimination of specific symptoms and detection of autoantibodies can distinguish CTD from IPF. However, there is growing evidence of substantial overlap, such that patients with IIP may also have signs and symptoms of CTDs including autoantibodies, arthralgias, skin eruptions, or photosensitivity, without fulfilling the criteria for a specific CTD.…”

Immunosuppressive and cytotoxic pharmacotherapy for pulmonary disorders.

“…The use of cytotoxic agents has escalated dramatically, even though firm data supporting their use may be lacking. These agents have been used as therapy for pulmonary vasculitis (10)(11)(12)(13)(14), chronic interstitial lung disorders (e.g., idiopathic pulmonary fibrosis, interstitial pneumonitis/fibrosis associated with rheumatic disorders, sarcoidosis) (1-7, [15][16][17][18][19][20][21][22][23][24][25][26], acute paraquat intoxication (27), and, more recently, asthma (28)(29)(30).…”

“…Nonetheless, as a practical approach, cytotoxic or immunosuppressive agents are often administered in IPF patients to avert deterioration and progressive respiratory failure (15)(16)(17)(18)(19)(20)(21). Similar treatment strategies have been adopted in nonrandomized clinical trials for interstitial pneumonitis/fibrosis complicating rheumatic disorders (23,(34)(35)(36)(37)(38). Diverse immunosuppressive agents have been used to treat corticosteroid-recalcitrant sarcoidosis (2,24,26,39), but randomized controlled studies have not been done.…”