Abstract
Introduction
Sarcoidosis is a multisystem granulomatous disease of unknown etiology, occasionally presenting with signs and symptoms that occur within the head and neck. Recently, granulomatous reactions and cases of sarcoidosis have been reported in patients treated with anti-TNF agents.
Methods
This report describes a 56-year-old man who developed sarcoidosis in the hypopharynx during adalimumab therapy for psoriatic arthritis. A retrospective review of the literature was performed using the PubMed database.
Results
In our patient, a chronic granulomatous reaction consistent with sarcoidosis developed after 2 years of continuous treatment with adalimumab. The diagnosis of sarcoidosis was established by the typical well-formed non caseating granulomas on biopsy, after excluding all other granulomatous conditions. Following withdrawal of anti-TNF agents and a course of steroids, the clinical picture resolved.
Conclusions
The development of sarcoidosis during treatment with TNF-a antagonists represents a rare and paradoxical adverse event. To our knowledge this is the first case of sarcoidosis of the hypopharynx reported in the literature.
1
Introduction
Sarcoidosis is a chronic inflammatory systematic disorder of unknown etiology with both pulmonary and extrapulmonary manifestations. Lung involvement is the predominant clinical feature; however, it can affect almost any organ .
It is typically characterized by non-caseating granulomas that can virtually develop in any organ . The disease was first described in 1875 by Hutchinson, but it was not until 1899 that Boeck reported the histological appearance of the lesions—hence it is also known as Boeck’s Sarcoid . The diagnosis is based on clinical presentation, histopathological findings and exclusion of other granulomatous disorders .
A growing number of reports concerning the development of granulomatous diseases as a result of paradoxical adverse effects of therapy with tumor necrosis factor (TNF) blocking agents have been reported in the literature .
TNF inhibitors are efficacious for treating rheumatoid arthritis and various other chronic inflammatory diseases . TNF plays a key role in the pathogenesis of sarcoid granulomas, along with other pro-inflammatory cytokines. It is a mediator of the molecular cascade that leads to chronic inflammation through the induction of chemokine production .
Treatment with monoclonal anti-TNF antibodies (infliximab, adalimumab) has been recently investigated for treating also refractory sarcoidosis and has been proven to be effective as they reduce inflammation by altering the human immune response . Therefore, the development of sarcoidosis in patients treated with these agents represents an unexpected adverse event .
2
Case report
A 66 year old man presented to our department with a two-month history of hoarseness and dysphagia. He also complained of a small ulcer on the left side of his tongue. His past medical history consisted of coronary artery disease for which he had undergone coronary artery bypass surgery 20 years earlier. He was also suffering from psoriatic arthritis that had been diagnosed by skin biopsies and a bone scintigraphy. He was on therapy with antihypertensive, anticoagulant and antihyperlipidemic agents. Since his psoriatic arthritis had been poorly responsive to conventional treatment regimens, he had also started therapy with adalimumab 6 years ago that had improved his symptoms.
Clinical examination of the oral cavity revealed a small ulcer on the left side of his tongue that was relatively soft, well circumscribed and had no signs of inflammation ( Fig. 1 ).
Flexible endoscopy revealed edema of the postcricoid area and the aryepiglottic folds and a lesion in the left pyriform sinus ( Fig. 2 ). Reduced mobility of the left vocal cord and sielostasis were also noted.
There was no evidence of regional lymphadenopathy.
The patient was admitted for further investigation and treatment of a suspected tumor of the hypopharynx. Routine hematologic and biochemical studies were normal. A chest radiograph was also within normal limits.
A computed tomography of the head and neck revealed a large soft tissue lesion on the left side of the hypopharynx, the oropharynx and the supraglottic area, pushing forward the left arytenoid ( Fig. 3 ). Marginally enlarged jugular lymph nodes were also present in the left side of the neck.
Computed Tomography of the thorax and abdomen was within normal limits apart from limited bronchiectasis in the upper lobe of the left lung.
The patient underwent a microlaryngoscopy and biopsy under general anesthesia that revealed a big edematous enlargement on the left hemilarynx. A hard mass with irregular surface was discovered on the postcricoid area, on the front wall of the entrance of the esophagus. Several biopsies were taken. At the same time, the ulcer of the left lateral side of the tongue was removed and sent for histopathologic examination.
Routinely Hematoxylin-and-eosin-stained sections of the biopsied material showed numerous epithelioid, non-necrotic granulomas with giant cells formation at the lamina propria in both sites ( Fig. 4 A ). Granulomatous reaction was also found between striated muscle fibers ( Fig. 4 B). The covered epithelium showed focal hyperplasia and ulceration. The histochemical Ziehl–Nielsen stain for acid-fast bacilli was negative. The findings were compatible with a chronic granulomatous inflammation such as sarcoidosis.
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