Uveoparotid fever, also known as Heerfordt-Waldenstrom syndrome, is a rare manifestation of sarcoidosis. The clinical presentation encompasses enlargement of the parotid gland (often bilateral), anterior uveitis, fever, and palsy of either the facial or trigeminal nerve. This syndrome is very rare in clinical practice, but through a detailed clinical investigation, the diagnosis is made solely on the presence of characteristic symptoms. The exact pathogenetic pattern connecting sarcoidosis and Heerfordt-Waldenstrom syndrome still remains to be fully understood.
So far, reports show that uveoparotid fever affects only about 0.3% of all patients suffering from sarcoidosis [1]. Furthermore, very few patients with uveoparotid fever have been described in the literature, but the clinical picture is well-established [2] [3] [4] [5]. Designated as an atypical clinical manifestation of sarcoidosis, uveoparotid fever presents with the following signs: parotid gland enlargement, anterior uveitis, fever, and cranial nerve palsy [1] [2] [3] [4] [5] [6]. Involvement of the parotid gland is rare and often develops in a bilateral fashion, which is also the case for uveitis, manifesting as blurred vision [2] [3]. Some authors also describe various forms of uveitis, such as panuveitis, granulomatous uveitis, myodesopsia, all frequently accompanied by eyelid swelling [5]. Fever is virtually always present and is one of the main symptoms seen in typical sarcoidosis (along with respiratory symptoms, joint pain, and weight loss) [1] [2] [3] [6]. Cranial nerve palsy predominantly targets the facial nerve either unilaterally or bilaterally (a facial droop is usually observed) but cases with a trigeminal nerve palsy have also been reported [1] [2] [3] [5] [6]. In addition, earlier reports noted weight loss and night sweats as features for uveoparotid fever, but these manifestations haven't been recognized in a significant number of individuals in more recent publications [5] [6]. Uveoparotid fever, generally diagnosed in patients between the second and fourth decade of life, is generally viewed as a self-limiting condition [1], but an early diagnosis undoubtedly facilitates better therapeutic response.
Because of the rarity of uveoparotid fever, the diagnosis may be difficult to make, especially if the primary symptoms of sarcoidosis are absent. For this reason, the physician must conduct a thorough clinical assessment, starting with detailed patient history, that will cover the onset of symptoms, duration, as well as progression. During the physical examination, neurological testing and a thorough ear, nose, and throat (ENT) exam can identify the main signs of uveoparotid fever - parotid gland enlargement, cranial nerve palsy, and uveitis. Thus, in the absence of specific tests, the diagnosis is predominantly made on clinical grounds [2] [4] [5] [6]. To confirm sarcoidosis as the underlying cause of symptoms, several tests need to be employed. High values of angiotensin-converting enzyme (ACE) is a highly useful finding during laboratory workup and it is assumed that granulomatous inflammation induces the production of this enzyme [2] [6]. If sufficient evidence is obtained, a lymph node biopsy should be conducted and a histological examination showing non-caseating granulomatous inflammation yields a definite diagnosis of sarcoidosis and uveoparotid fever [2] [5] [6].