By Ashley Henderson MD
A 52 year old woman with stage IV sarcoidosis and known apical
fungus ball presented with massive hemoptysis (>200 cc blood in 24 hours).
She had had a recent upper respiratory tract infection, but was no longer
having a productive cough when she noticed "fullness" on the right
side of her chest and then coughed up bright red blood.
Her past medical history is significant for sarcoidosis diagnosed
in 1992 by skin biopsy. She subsequently developed pulmonary involvement that
has required immunosuppressants to maintain control of her cough and adequate
lung function. Despite aggressive treatment, she progressed to stage IV sarcoidosis
over four to five years. In addition, approximately 9 months prior to admission
she had developed rare blood-streaked sputum, which resulted in a CT scan
demonstrating a developing fungus ball in her right apex. Because of this,
she was started on itraconazole in hopes of controlling growth of the fungus
ball. She also has a history of mild gastroesophageal reflux disease.
Her current medications are prednisone 10 mg/day, methotrexate
20 mg/wk, itraconazole 200 mg BID, inhaled fluticasone 220ug BID, inhaled
salmeterol BID, omeprazole 40 mg/day, vitamin D and calcium. There are no
other family members with sarcoidosis.
Her physical exam was notable for tachycardia, temperature of 39.8 C, BP 102/76, RR 20 with oxygen saturations of 99% on 2L of oxygen, normal breath sounds throughout and normal cardiac sounds. Skin was moist and no evidence of lesions. Neurologic exam was non-focal. She was admitted and scheduled for emergency bronchial artery embolization, but she resolved her hemoptysis while in the emergency room. She was started on IV antibiotics, had no further hemoptysis and was discharged on hospital day 5 to complete a course of antibiotics.
This case provides for several important problems to consider.
The first issue is the role of bronchial artery embolization in patients with
sarcoidosis. This procedure has been shown to be helpful for patients with
cystic fibrosis and occasionally helpful in patients with malignancy, but
its role in treatment of hemoptysis with fungus balls is unclear. However,
it can be a lifesaving procedure in the face of non-remitting severe hemoptysis
in these patients, and is often used for treatment. The long-term data show
that it is temporizing, but it controls an acute situation. (1)
Another concern is the treatment of fungus balls. This patient
has shown progression of disease despite chronic treatment with oral anti-fungal
agents, which is not uncommon, but she is now having significant symptoms
from this mycetoma. However, she is also on chronic immunosuppressive therapy
that might contribute to her risk of having fungus balls and/or progression.
. When possible, it is best to decrease or remove the amount of immunosuppressants
given, which have done in this patient.
Finally, this patient has progressive respiratory disease, but
current literature states that the risk of lung transplantation is higher
in these patients secondary to the risk of fungemia post-operatively with
the necessary high-dose immunosuppressants given. Some institutions are having
improved results secondary to more aggressive anti-fungal therapy (2), but
there are still many institutions that will not transplant these patients.
Because of these concerns, one potential treatment is intracavitary injection
of amphotericin. Recent data suggests that this procedure may not only improve
the hemoptysis, but also significantly delay the recurrence of the fungus
ball. Although long-term data is still unknown, removing a life-threatening
problem of hemoptysis temporarily may be beneficial in this patient. (3)
If unable to resolve the hemoptysis with intracavitary therapy, the definitive
therapy is surgical resection. (1)
Bibliography:
1. Stevens DA, Kan VL, Judson MA, Morrison VA, Dummer S, Denning
DW, Bennett JE, Walsh TJ, Patterson TF, Pankey GA. Practice guidelines
for diseases caused by Aspergillus. Infectious Diseases Society of America.
Clinical Infectious Diseases. 2000 Apr; 30(4):696-709.
2. Hadjiliadis D, Sporn TA, Perfect JR, Tapson VF, Davis RD, Palmer SM. Outcome
of lung transplantation in patients with mycetomas. Chest 2002
Jan;121(1):128-34
3. Giron J, Poey C, Fajadet P, Sans N, Fourcade D, Senac JP, Railhad JJ. CT-Guided
Percutaneous treatment of inoperable pulmonary aspergillomsa: a study of 40
cases. European Journal of Radiology 1998 28:235-242.