Isolated Chronic Dacryocystitis: A rare presentation of Sarcoidosis

Summary: Isolated Chronic Dacryocystitis: A rare presentation of Sarcoidosis Alsayed Osman MD, Noor Bazerbashi MD, Robert Ryad MD, Yih Chang Lin MD. Department of Internal Medicine, Division of Rheumatology, University of South Florida. Keywords: Dacryocystitis, Non-caseating granuloma, Sarcoidosis Background: Sarcoidosis is a chronic systemic granulomatous disease of unknown etiology, but isolated lacrimal gland involvement is rare. We report a case of Sarcoidosis presenting as isolated chronic dacryocystitis, without systemic features. Case description: A 69-year-old female experienced chronic swelling of the left eyelid for six months. She denied symptoms such as fever, chills, parotid enlargement, arthritis, facial nerve palsy, or uveitis. Her review of systems was unremarkable for chronic dyspnea, cough, chest pain, skin rash or panniculitis. Upon examination, the patient had swelling and erythema of the left upper eyelid, but her extraocular muscles movement was intact. She had previously been treated with antibiotics and steroids without sustained improvement. After lacrimal duct irrigation proved ineffective, she underwent dacryocystorhinostomy with lacrimal stent placement, along with left lacrimal gland biopsy. The biopsy revealed non-caseating granuloma, suggesting sarcoidosis. Staining for fungal elements (GMS), acid-fast bacilli (AFB), and Fite stains were negative. Immunoglobulin G4 (IgG4) staining showed rare plasma cells. The work-up showed normal calcium, 25 hydroxyvitamin D, and 1,25 hydroxyvitamin D. The Angiotensin Converting Enzyme (ACE) level was low. The patient tested negative for QuantiFERON-TB Gold (QTG), Antinuclear Antibody (ANA), and Rheumatoid Factor (RF), with normal IgG subclasses and complement levels. Chest X-ray showed no hilar lymphadenopathy. Electrocardiogram (EKG) and echocardiogram results were unremarkable. The patient responded initially to intravenous methylprednisolone, but symptoms worsened when transitioning to oral prednisone. A switch to oral methylprednisolone led to significant improvement, prompting treatment with Methotrexate as a steroid-sparing agent. The lacrimal stent was removed after three months without recurrence of swelling. Discussion: Sarcoidosis typically presents with multisystem involvement, but isolated dacryocystitis can be a rare initial manifestation. Given the finding of non-caseating granulomas in this patient, sarcoidosis was considered, even in the absence of systemic features. It is crucial to rule out other causes like Immunoglobulin G4-related disease (IgG4-RD), granulomatosis with polyangiitis (GPA), fungal, and mycobacterial infections in this population. Conclusion: Isolated sarcoidosis of the lacrimal sac is rare and can be challenging to diagnose. A thorough evaluation is necessary when granulomatous findings are identified. This case underscores the importance of considering sarcoidosis in differential diagnoses, even without classic systemic symptoms.