Abstract
Involvement of the larynx and nasopharynx in sarcoidosis is rare. Its course, like other forms of sarcoid, is one of remission and relapse usually responding to high-dose steroids or other forms of immunosuppression. We report a patient with nasopharyngeal and laryngeal sarcoid who failed to respond to immunosuppression but was treated effectively with low-dose radiotherapy. A 39-year-old Afro-Caribbean man presented in November, 1995, with reduced visual acuity. There was no medical or family history of sarcoidosis. Examination confirmed bilateral panuveitis. There were multiple cutaneous nodules on his limbs. Sarcoidosis was confirmed by skin biopsy, a raised serum angiotensin converting enzyme concentration of 109 IU/L, bilateral hilar lymphadenopathy on chest radiography, and characteristic lung function tests (FEV1 3·57 L, FVC 4·08 L, FEV1/FVC 88%, TLCO 4·8 mmol/min per kPa, predicted values FEV1 4·02 L, FVC 4·8 L, FEV1/FVC 84%, TLCO 11·13 mmol/min per kPa). His panuveitis responded to oral prednisolone 60 mg once daily and his lung function tests improved. Prednisolone was reduced to 20 mg once daily over 5 months with no relapse. However, 5 months after presentation, he developed nasal congestion and stridor. Indirect laryngoscopy revealed multiple nasopharyngeal and laryngeal nodules. A biopsy sample showed non-caseating granuloma consistent with sarcoidosis. Despite increasing prednisolone to 60 mg daily and adding cyclosporin, 400 mg once daily for 4 weeks, with satisfactory therapeutic blood concentrations, his nasal congestion and stridor progressively worsened. Cyclosporin was stopped, and on the basis of several successful reports of radiotherapy in steroid refractory neurosarcoid, we decided to treat him with low-dose external beam radiotherapy to his nasopharynx and larynx (20 Gys in 10 fractions over 2 weeks). He had complete remission of his symptoms at 7 weeks and they had not returned 12 months after radiotherapy. His present medication is prednisolone 15 mg once daily. This report is unusual in that sarcoidosis of the nasopharynx and larynx failed to respond to immunosuppressants but was successfully treated by irradiation. We do not advocate the routine use of radiotherapy for the treatment of this form of sarcoidosis on the basis of this single report, but suggest that it may be an alternative treatment in patients with life-threatening disease refractory to conventional immunosuppressant therapy.
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