Carpenter syndrome

Muhammad Shibli MD, J Ghassibi MD, R Hajal MD, M O'Sullivan MD--St. Luke's/Roosevelt Hospital Center, New York, NY

Introduction: Coccidioidomycosis is a fungal infection that usually causes a benign self-limited upper respiratory infection in endemic areas, and rarely causes a fulminant disseminating illness in immunocompetent patients(1). Acute respiratory distress syndrome (ARDS) with severe gas exchange impairment is a rare complication that is associated with high mortality(2). Corticosteroids use has generally been avoided iii patients with evidence of fungal infection. We are presenting a case of severe ARDS due to a fulminant coccidioidomycosis infection that responded to a short course of systemic steroids in addition to the standard antifungal treatment.

Case Presentation: 23 year-old African American man, non-smoker, was admitted with worsening back pain, 10 pounds weight loss, high-grade fever, dry cough and dyspnea on exertion of two months duration.

He was a soldier in the US Army with extensive travel history to San Diego, CA. North Carolina, South Carolina, Alabama, Europe and South America. His physical examination was remarkable for bilateral coarse breath sounds and a tender right gluteal area. Initial work up revealed a leukocyte count of 18,500 with 17.6% eosinophils, diffuse bilateral nodular infiltrates on chest x-ray, diffuse reticulo-nodular pattern on CT scan of the chest and a lobulated mass at the head of the 12th rib. He also had multiple vertebral lesions of increased intensity on MRI. Biopsy of the lobulated mass revealed chronic inflammation and many spherules consistent with coccidioides immitis. His HIV titer was negative. He was started on intravenous amphotericin B and later fluconazole was added for persistent fever. His hospital course was complicated on day 16 by a progressive respiratory distress and severe hypoxemia requiring endotracheal intubation. During his ICU stay, his pulmonary infiltrates and his hypoxemia worsened. He required a PEEP of 10 cm H20 and an FiO2 of 0.8. In light of the worsening in oxygenation the decision was made to start him on intravenous methylprednisolone 60 mg daily. Within a day his fever subsided. Two days later his oxygenation improved. The methylprednisolone was reduced to 40 mg once a day. His oxygenation and overall condition continued to improve and eventually a 14 days course of systemic corticosteroids was completed. He was extubated 6 days after the start of methylprednisolone and transferred the following day out of the ICU. He remained afebrile and was discharged on hospital day 52 on suppressive oral Fluconozole.

Discussion: The beneficial role of adjunctive corticosteroids have been demonstrated in miliary tuberculosis, tuberculous meningitis and pneumocystis carinii pneumonia(3). Although their exact mechanism of action in these situations is unclear, it is believed that corticosteroids modulate the host inflammatory reaction to the offending organism (3,4). We believe that a similar mechanism occurred in this patient where the disseminated infection resulted in severe pulmonary inflammation associated with severe impairment of gas exchange. There was a dramatic improvement in this patient's oxygenation within two days of corticosteroids administration most probably related to the control of the host inflammatory response reaction. Moreover, the use of steroids in this patient was not associated with any complications related to immunosuppression.

Conclusion: The usual practice is to avoid corticosteroid therapy in patients with fungal infections. However, we were able to shove a beneficial and a significant response to adjunctive corticosteroids in a patient with ARDS due to disseminated coccidioidal infection. It might be safe to add steroids to patients with severe inflammatory reaction even in the presence of fungal infection as long as the appropriate anti-fungal treatment is being administered.

References

(1) Stevens DA. Coccidioidomycosis. NEJM 1995; 332(16):1077-82

(2) Arsura E, Kilgore W. Miliary coccidioidomycosis in the immunocompetent. Chest 2000; 117(2):404-09

(3) Dooley D, Carpenter J, Rademacher S. Adjunctive corticosteroid therapy for tuberculosis. Clin. Inf. Dis 1997; 25:872-87

(4) Jantz M, Sahn S. Corticosteroids in acute respiratory failure. Am J Respir Crit Care Med 1999; 160:1079-1100

COPYRIGHT 2000 American College of Chest Physicians
COPYRIGHT 2001 Gale Group