Baylor University Medical Center Proceedings October 2017 - 453

Figure 2. CT of the neck revealing (a) thick nasal lining with obstructed nasal vestibules (arrow); (b) bilateral maxillary erosions (arrow); and (c) destruction of the
premaxilla, palate, and floor of the nose and complete opacification of the left maxillary sinus (arrow).

for worsening shortness of breath, nasal obstruction, and stridor.
Oral prednisone was diﬃcult to reduce due to rebounds in her
respiratory symptoms. A rheumatologist then started her on a
weekly subcutaneous injection of methotrexate 25 mg and oral
hydroxychloroquine 400 mg daily. She reported improvement of
nasal obstruction and stuﬃness. Two months later, subcutaneous injections of 250 mg (2 mg/kg) of golimumab (Simponi®,
Janssen Biotech, Horsham, PA) were started. She reported more
improvement in her nasal symptoms and shortness of breath but
no change in her hoarseness. The prednisone dose was decreased
to 10 mg daily, and she has not recently required hospitalization.
DISCUSSION
Sarcoidosis is a chronic multisystemic disease of unclear
etiology; it has a prevalence of 10 to 20 per 100,000 persons in
the United States and is more common in women (1). Sarcoidosis typically aﬀects patients under 40 years, with a peak among
those in their 20s. The clinical course of sarcoidosis is variable;
60% to 70% of patients have a spontaneous remission, and
30% of patients have prolonged courses of more than 5 years.

Figure 3. Nasal biopsy demonstrating a granuloma next to a hair follicle in the
dermis, which has an extensive inflammatory infiltrate (hematoxylin and eosin, 200×).
October 2017

The mortality rate is 1% to 5% and is mostly related to cardiac,
pulmonary, and neurological complications (2).
Upper airway disease in sarcoidosis is uncommon and occurs
in <2% of patients with sarcoidosis. Multiorgan involvement is
uncommon in these patient. Benjamin et al reported that out of
5 patients with laryngeal involvement, only one had generalized
disease (3). Neel et al found that only 7 of 13 patients with laryngeal sarcoidosis had other organ involvement (4). The clinical
presentation of upper airway sarcoidosis varies from asymptomatic
to severe. The main symptoms of sinonasal disease include nasal
obstruction, crusting, and epistaxis (5). Laryngeal disease presents
with hoarseness, dyspnea, dysphagia, chronic cough, obstructive
sleep apnea, and airway obstruction, which could progress to upper
airway obstruction and emergency cricothyrotomy (6). Fiberoptic
nasolaryngoscopic examination of the nasal mucosa of patients
with sinonasal sarcoidosis typically reveals pale, yellow nodular
lesions and inﬂammation with mucus crusting (7). Our patient
had these ﬁndings.
In laryngeal disease, the supraglottis region, mostly the epiglottis, is typically involved, followed by the arytenoids, aryepiglottic
folds, and false vocal folds (8). The subglottis can be involved in
24% of the cases of laryngeal sarcoidosis. However, the glottis
is very rarely aﬀected (9). Flexible ﬁberoptic laryngoscopy often
shows pale pink, edematous, and nodular thickening of the epiglottis, arytenoids, and aryepiglottic folds, a classic pattern of supraglottic involvement. Vocal cord involvement can lead to dyspnea,
hoarseness, stridor, and immobility (9). Fiberoptic examination
in our patient revealed edematous and pink aryepiglottic and vestibular folds with a subglottic nodular lesion; no epiglottic lesion
was noted. Imaging with CT scans in sinonasal sarcoidosis reveals
turbinate or septal nodularity/thickening (21%), osteoneogenesis,
and bony erosions (15%-20%) (10). It is important to exclude
other granulomatous diseases, such as fungal infections and polyangiitis with granulomatosis. Direct bone involvement in sarcoidosis
is relatively uncommon and occurs in <15% of patients; rarely the
disease can extend from sinuses into intracranial structures (11).
Sarcoidosis of the upper respiratory tract is associated with
lower rates of spontaneous remission and often requires systemic

Sinonasal and laryngeal sarcoidosis

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Table of Contents for the Digital Edition of Baylor University Medical Center Proceedings October 2017