Cryptogenic Organizing Pneumonia (COP) can present with respiratory failure with bilateral infiltrates. Unilateral disease has not been described in this setting.
A previously healthy 37 year old housewife with with a 21 pack year history of smoking, presented with several weeks of increasing dyspnea, cough, mild hemoptysis, fever, and wheezing after failing antibiotics and steroids.. Her medications included salmeterol/ fluticasone 250mg/ 50mg twice daily, prednisone 20mg daily (as part of a tapering regimen), and levofloxacin 500mg daily. On exam:BP 127/75 mm Hg, HR 96, respiration rate 20, T 36.3 degrees Celsius, and oxygen saturation of 77% on 3 L per minute nasal cannula. General exam revealed a tachypneic female with some crusted blood in her left nare. The patient’s neck was supple with no palpable lymphadenopathy with flat jugular veins. Scattered inspiratory and expiratory wheezes were auscultated bilaterally. Her cardiac exam revealed a short systolic murmur and a prominent P2. The patient’s abdomen was distended with minimal hepatosplenomegaly. There was trace pretibial edema and peripheral cyanosis without clubbing. The patient’s blood work revealed a WBC 19,200 with 88% polymorphonuclear and 4% lymphocytes. Chemistry results were within normal limits. Chest radiograph revealed a diffuse right sided infiltrate. A chest CT demonstrated diffuse crazy paving in the right lung with a small pleural effusion, left upper lobe linear opacities, and diffuse mediastinal, subcarinal, and hilar lymphadenopathy. The patient was admitted for respiratory failure and treated with broad spectrum antibiotics and oxygen therapy. Despite aggressive antibiotics, the patient required 100% oxygen without improvement. Her lab work, including cultures remained negative. The patient underwent an open lung biopsy and she required mechanical ventilation after that. Multiple wedge biopsies were performed from the right lung which revealed a patchy temporally homogenous process with intraluminal plugs of fibrosis with a relative preservation of lung architecture. No signs of necrosis, neutrophilic or eosinophilic inflammation, granuloma or foreign material, hyaline membrane formation, evidence of vasculitis or malignancy were observed.
COP is the idiopathic organizing pneumonia and is typically considered a diagnosis of exclusion. Organizing pneumonias can be associated with connective tissue diseases, several medications, malignancy, and other interstitial pneumonias. COP typically affects men and women equally in the 5th to 6th decades of life. Patients typically present with fatigue, malaise, nonproductive cough, fever, and dyspnea with exertion. Functionally, COP is characterized by a restrictive ventilatory defect, a reduced diffusing capacity, and hypoxemia. Consolidation is the primary observation on chest radiographs and CT scans of patients with COP. The consolidation is frequently bilateral, and is usually patchy and asymmetric. Other patterns include peripheral lobar or segmental alveolar infiltrate with air bronchograms or diffuse reticulonodular infiltrates. The radiographic opacities may be migratory. Nodules and irregular linear opacities may be seen in 10%to 40% of cases. Several case reports indicate COP can uncommonly present as an acute fulminant illness leading to respiratory failure. This manifests as bilateral pulmonary opacities. Early steroid treatment is necessary. Our patient’s presentation was unique in that she had a fulminant course from COP with a unilateral infiltrate involving the entire right lung.
A high index of suspicion for COP is necessary in acute respiratory failure even with a diffuse unilateral process to improve outcomes.
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