Answer: C. Hypersensitivity Pneumonitis
Figure 1. Subtle scattered nodules and ground glass opacities mainly in the upper lobes.
Figure 2. Ground glass opacities involving the upper lobes and lower
(not pictured), with subpleural sparing and mosaic attenuation.
Figure 3. More consolidation with sub-pleural sparing in the upper lobes and mosaic attenuation in the lower lobes.
Figure 4. Pathologic findings: A. Airway centered lymphocytic
infiltration with thickening of the alveolar septa (arrows) and loose
granuloma (arrowhead) (hematoxylin-eosin, 100x); B. Vaguely formed
nonnecrotizing granulomata in the interstitium (arrowheads) with
fibrosis (arrows) (hematoxylin-eosin, 40x); C. Peribronchiolar giant
cells with poorly formed granulomas and mononuclear infiltration
(hematoxylin-eosin, 200x).
Discussion
Upon further interview, our patient admitted to having a pet-bird for
years. These radiologic and histopathologic findings along with a
history of exposure to organic antigens in a symptomatic patient are
consistent with the diagnosis of subacute hypersensitivity pneumonitis
(HP). The patient was started on steroids and relocated her pet bird.
Four months later, her symptoms of cough and dyspnea were significantly
improved.
Subacute HP is a diffuse granulomatous interstitial lung disease
caused by inhalation of various antigenic organic particles. Repeated
antigen exposure causes immunologic sensitization of the host to the
antigen and immune-mediated damage to the lung. These antigens are
presented by macrophages and dendritic cells, resulting in the
differentiation of CD4 lymphocytes towards Th1 cells. Cytokines are
secreted by Th1 lymphocytes and macrophages which promote granuloma
formation. There is interstitial and peribronchiolar lymphocyte
accumulation.
Patients usually present with insidious onset of dyspnea, fatigue and
cough over weeks to months. Pulmonary function tests reveal
restrictive pattern and reduction of the carbon monoxide diffusion
capacity (DLCO). Bronchoalveolar lavage findings include an
increase in the total cell count with a marked lymphocytosis. The
CD4+/CD8+ ratio is classically less than 1. Options for tissue sampling
include transbronchial lung biopsy, transbronchial cryobiopsy and
surgical lung biopsy with a diagnostic yield of 64, 84 and 91%
respectively. Histopathologic findings usually consist of
bronchiolocentric lymphohistiocytic interstitial pneumonia, chronic
bronchiolitis and small, poorly formed, nonnecrotizing granulomas.
Differential diagnosis of CT findings of ground glass opacities
includes: 1) Non-specific interstitial pneumonia: in this case presence
of centrilobular nodules make it less likely; 2) Cryptogenic organizing
pneumonia: although ground glass opacities may be present, patchy
consolidation especially in subpleural areas is a dominant feature; 3)
Desquamative interstitial pneumonia: ground glass opacities are usually
basilar or subpleural and more than 90% patients are smokers which is
not consistent with this case.
Diagnosing ILD requires a high index of clinical suspicion,
particularly for HP. A thorough history of exposure to antigens at
home, work, or related to hobbies must be obtained. Removal of the
causative agent, if possible, remains the cornerstone of management.
Randomized controlled trials are lacking for all the
pharmacotherapeutic agents used for treatment of HP. Systemic
corticosteroids are used most often with the aim for lowest-possible
dose and shortest duration. Immuno-modulating agents (e.g.
azathioprine, mycophenolate) and lung transplantation may be considered
in patients with progressive disease. The presence of fibrosis at lung
biopsy or HRCT scan is a poor prognostic sign.
References
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