Granulomatous Respiratory Disorders
Paul C. Stillwell, MD, FAAP, and Robin R. Deterding, MD
Introduction/Etiology/Epidemiology
•Granulomatous lung disease is categorized on the basis of several features of the granulomas, such as caseating and noncaseating and infectious or noninfectious etiologic origin (Boxes 91-1 and 91-2).
•The common feature is the pathologic appearance of an aggregate of histiocytes that are elongated and have indistinct borders.
—Multinucleated giant cells, lymphocytes, and plasma cells may also be present.
—If central necrosis is present, the granuloma will appear to have caseation and is most commonly caused by an infection (Figure 91-1).
•Granulomatous lung diseases are often associated with granulomas in several other organ systems, including the skin, subcutaneous tissues, and lymph nodes.
•Notable causes of granulomatous lung disease include
—Sarcoidosis
▪A multisystem disease of unknown etiologic origins
▪Noncaseating granulomas are the hallmark pathologic finding.
Box 91-1. Common Infectious Causes of Granulomatous Lung Disease in Children
Mycobacteria
Mycobacterium tuberculosis
Nontuberculous mycobacteria
Fungus
Histoplasma capsulatum
Blastomyces dermatitidis
Coccidioides immitis
Cryptococcus
Parasites
Dirofilaria (rare)
Toxoplasma gondii
Box 91-2. Common Noninfectious Causes of Granulomatous Diseases in Children
Sarcoidosis
Hypersensitivity pneumonitis
Bird fancier disease
Farmer lung
Hot tub lung
Vasculitis
Granulomatosis with polyangiitis (formerly Wegener granulomatosis)
Eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome)
Aspiration (chronic)
Immunodeficiency
Chronic granulomatous disease
Common variable immunodeficiency (granulomatous lymphocytic interstitial lung disease)
Hypogammaglobulinemia
Lymphoid interstitial pneumonitis
Crohn disease
Bronchocentric granulomatosis (often associated with Aspergillus)
▪Pulmonary involvement occurs in approximately 85% of adult patients; it may be slightly less common in children, particularly those <5 years of age.
▪Other organ systems commonly affected include the eyes, reticuloendothelial system (liver, lymph nodes, and spleen), skin, heart, and central nervous system.
▪In the United States, sarcoidosis is more prevalent and more severe in the African American population.
—Granulomatous lymphocytic interstitial lung disease (GLILD) associated with common variable immunodeficiency has been recognized with increasing frequency and, in some areas, is more common than sarcoidosis.
—Other causes include mycobacterial tuberculosis (see Chapter 58, Tuberculosis), nontuberculous mycobacterial lung disease (see Chapter 59
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