Page 1012 - Clinical Immunology_ Principles and Practice ( PDFDrive )

976 Part Seven Organ-Specific Inflammatory Disease

TABLE 72.3 Disorders associated With a TABLE 72.4 Pleuropulmonary
Bronchiolitis Obliterans Organizing Manifestations of Connective tissue
Pneumonia (BOOP) Pattern Diseases

Idiopathic BOOP (cryptogenic organizing pneumonia) SLe ra SSc
Connective tissue diseases
• Systemic lupus erythematosus Pulmonary hypertension + + +++
• Rheumatoid arthritis Vasculitis + ± ±
• Polymyositis/dermatomyositis Pleural disease +++ +++ +
• Sjögren syndrome Bronchiolitis obliterans ± ++ +
Hypersensitivity pneumonitis Aspiration pneumonia − − ++
Chronic eosinophilic pneumonia Diaphragmatic dysfunction ++ − −
Drug-induced Lung nodules − ++ −
• Gold Diffuse alveolar damage + ± ±
• Penicillamine BOOP ± + ±
• Amiodarone UIP + ++ +
• Bleomycin Capillaritis ++ + ±
• Sulfa drugs LIP + + +
Granulomatosis with polyangiitis (wegener granulomatosis) NSIP + ++ ++
Bone marrow transplantation BOOP, bronchiolitis obliterans organizing pneumonia; LIP, lymphocytic interstitial
Lung transplantation/rejection pneumonia; NSIP, nonspecific interstitial pneumonia; RA, rheumatoid arthritis; SLE,
Inhalational injury systemic lupus erythematosus; SSc, systemic sclerosis; UIP, usual interstitial
neoplasms pneumonitis.
Lung irradiation
virus-associated
• Human immunodeficiency virus (HIV)
• Influenza virus mainly affects young women (female-to-male ratio >8 : 1) and
• Adenovirus may involve virtually every organ system. Pleuropulmonary
involvement occurs at some point in the disease course in 38–89%
34
of cases. Thus the respiratory system is affected more commonly
in SLE than in any other CTD. However, infectious pneumonia
remains the commonest cause of pulmonary disease and death
Treatment and Outcome in these patients. Thus in patients with SLE presenting with a
Treatment with glucocorticoids usually offers dramatic clinical febrile illness and pulmonary infiltrates, a community-acquired
33
and radiographic improvement within days to weeks. Complete or opportunistic infection must be promptly excluded.
clinical, physiological, and radiographic recovery occurs in two-
thirds of cases. In the remainder, persistent disease progresses Acute Lupus Pneumonitis
to fibrosis. It is common for relapses to occur with glucocorticoid Acute lupus pneumonitis is an uncommon pulmonary manifesta-
34
tapering, followed by improvement with reintroduction of tion of SLE, occurring in fewer than 5% of cases. The clinical
treatment; consequently at least 6 months of therapy is recom- presentation mimics that of an infectious pneumonia with the
mended. The 5-year survival rate in COP is 73%, compared with abrupt onset of fever, cough, and dyspnea. Serum complement
5-year survival rates of 44% in patients with BOOP resulting levels are often low, and chest radiography typically shows diffuse
from other causes (e.g., CTD) or 30% for IPF. alveolar opacities. Acute lupus pneumonitis can be accompanied
by pericarditis and often pleuritis and pleural effusion.
LUNG INVOLVEMENT IN CONNECTIVE It can be difficult to distinguish acute lupus pneumonitis
TISSUE DISEASES from an infectious pneumonia. BAL followed by thoracoscopic
lung biopsy is recommended before instituting corticosteroid
CTDs are a heterogeneous group of systemic autoimmune diseases therapy. The histopathology varies and includes diffuse alveolar
that frequently involve the lungs. The pleuropulmonary mani- damage, BOOP, NSIP, or a combination of these.
festations of these diseases are diverse, affecting all parts of the There are no controlled trials of therapy for acute lupus
respiratory tract (i.e., airways, alveoli, blood vessels, and pleura) pneumonitis. Treatment includes high-dose glucocorticoids
(Table 72.4). Although pulmonary complications generally occur (1–2 mg/kg/day) with or without accompanying cytotoxic drugs,
in patients with well-established disease, occasionally the lung such as cyclophosphamide. Mortality rates as high as 50% have
involvement is the first manifestation of the autoimmune disorder. been reported. In those patients who fail to respond to treatment,
This section discusses the pleuropulmonary manifestations of respiratory failure is the usual cause of death.
systemic lupus erythematosus (SLE), RA, and systemic sclerosis
(SSc) (for a discussion of other manifestations in these diseases, Diffuse Alveolar Hemorrhage
see Chapters 51, 52, and 55). DAH occurs in fewer than 5% of patients with SLE, and it
represents the initial manifestation of disease in 11–20% of those
Systemic Lupus Erythematosus cases. However, most cases develop in individuals with well-
34
SLE is a disease of unknown etiology characterized by the presence established diagnoses of SLE, usually with preexisting lupus
of autoantibodies directed against various nuclear antigens. These nephritis.
autoantibodies and the resultant immune complexes mediate The symptoms of DAH mimic those of infectious pneumonia
34
many of the manifestations of SLE (Chapter 51). This disease and acute lupus pneumonitis. Hemoptysis is present in 42–66%