Bronchiolitis Obliterans With Organizing Pneumonia and Cold Agglutinin Disease Associated With Phenytoin Hypersensitivity Syndrome: Discussion

In: Pulmonary function

10 Mar 2014

Bronchiolitis Obliterans With Organizing Pneumonia and Cold Agglutinin Disease Associated With Phenytoin Hypersensitivity Syndrome: DiscussionPHS is rare with few reports describing pulmonary involvement. Fever, cough, dyspnea, hypoxemia, and bilateral infiltrates on a chest x-ray film in the setting of a more generalized hypersensitivity reaction are typical. Herein is the first description of BOOP in PHS. BOOP is characterized by ingrowth of polypoid fibroinflammatory granulation tissue from bronchioles into adjacent alveoli where an organizing pneumonia forms. The infiltrate is predominantly mononuclear, patchy, and thought to be part of the lung’s reparative process after insults, including hypersensitivity reactions. Commonly, BOOP is sensitive to steroid therapy, associated widi a restrictive lung defect and decreased carbon monoxide diffusing capacity. X-ray film findings vary and often show multiple, patchy interstitial-alveolar infiltrates like those described here. Use of crack cocaine, implicated in one case of BOOP, was not thought significant here since this patient ceased cocaine use after his first hospital discharge and clearly suffered from a generalized hypersensitivity syndrome, a finding not associated with cocaine. tadanafil

Ill lino virus found on BAL culture was likely the result of upper airway contamination during bronchoscopy. The clear rhinorrhea at illness onset, multiple other case reports suggesting concomitant respiratory tract infections, and the reported seasonal variation of phenytoin hypersensitivity reactions suggest a role for concurrent infection in triggering or intensifying the syndrome or, conversely, infections may be the result of phenytoin’s effect on immune function. Sepsis-like hemodynamics, responsive to steroids, have been described rarely in PHS.
Coombs-negative hemolytic anemia has been repeatedly noted in PHS. Coombs’-positive hemolysis, however, was mentioned only once in the present review of the literature. Here is provided the first full description of acute cold hemagglutinin disease in PHS. Clear evidence of new onset cold hemagglutinins with a positive direct antiglobulin test for complement only and presence of IgM anti-I autoantibodies of high enough thermal amplitude and titer to produce a clinical picture consistent with both intravascular and extravascular hemolysis were found. Such low-titer, high thermal amplitude-type cold agglutinins are responsive to high-dose steroids. Currently, no consensus exists regarding the optimal dose or treatment duration of corticosteroids for BOOP, nor are there guidelines for steroid use in PH S. This patient’s response to intravenously administered methylpred-nisolone, 60 mg every 6 h for 48 h with a slow intravenous taper over a week, was followed by the regimen suggested by Epler and Colby for BOOP: prednisone, 1 mg/kg for 3 months with a subsequent slow drug taper so that steroid therapy was used for a total of 12 months. This proved to be satisfactory for the patient reported herein.

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