A CASE OF INCOMPLETE HEERFORDT'S SYNDROME

Document Type

Conference Proceeding

Publication Date

6-17-2022

Publication Title

Journal of General Internal Medicine

Abstract

CASE: A 41-year-old male with no past medical history presented with 60 lbs. weight loss over a 6-month time interval, dry mouth, morning cough and night sweats. Pertinent family history consisted of a maternal cousin with sarcoidosis. Physical exam demonstrated a mild left facial palsy. Lab results revealed an elevated Angiotensin-1 Converting Enzyme (ACE) level of 71 U/L (range 8-52 U/L). He underwent a GI work up, which revealed enlarged abdominal lymph nodes. He was then referred to oncology for concern of occult malignancy. The patient received a PET/CT scan which demonstrated bilateral parotid gland enlargement, hilar adenopathy and reticulonodular involvement of the lungs. A bronchoscopy with endobronchial ultrasound, transbronchial needle aspiration and right-upper lobe lung transbronchial biopsy was performed, which displayed non-caseating granulomas. The patient was diagnosed with sarcoidosis. Considering the patient's history, physical exam, radiologic findings and biopsy results, he was further classified with Incomplete Heerfordt' Syndrome and started on prednisone 40 mg every day. At his follow-up appointment, the patient reported resolution of symptoms, 30 lbs weight gain and imaging demonstrated significant improvement. IMPACT/DISCUSSION: The inflammatory process of sarcoidosis can involve many organ systems of the body, making it a great masquerader of diagnostic medicine. Heerfordt's Syndrome is a rare manifestation of sarcoidosis, with a symptom triad of uveitis, parotid gland enlargement and facial nerve palsy. Complete Heerfordt's Syndrome is diagnosed when all 3 symptoms are present, accounting for 0.3% of sarcoidosis cases. Incomplete Heerfordt's Syndrome (2 of 3 symptoms present) accounts for 1.3% of sarcoidosis cases. We report a case of Incomplete Heerfordt's Syndrome in a middle-aged male who presented with vague symptoms. Given its rarity and ambiguous presentation, diagnosis of Heerfordt's Syndrome can be difficult. It is important to maintain a high clinical index of suspicion for Heerfordt's Syndrome as early detection, diagnosis and treatment can prevent long-term sequelae of chronic tissue inflammation and damage. Based on the presence of neurosarcoidosis, corticosteroid treatment has been implicated as initial treatment. Refractory treatment includes immunosuppressants such as azathioprine and methotrexate. CONCLUSION: It is important for a clinician to recognize the signs and symptoms of Heerfordt's Syndrome. Early diagnosis and treatment of Heerfordt's Syndrome can prevent irreversible tissue damage.

Volume

37

Issue

Suppl 2

First Page

S350

Find It @ Sladen

Share

COinS