Dacryoadenitis as a Rare Initial Presentation in a Patient with Suspected Crest Syndrome.

Abstract

Systemic sclerosis (SSc) is a rare, chronic disease with diverse clinical presentations, and only a few cases with ocular manifestations have been reported in the literature. In this case report, we describe the case of a 51-year-old South Asian woman who initially complained of painless swelling in her left upper eyelid. An ultrasound examination of the left eye revealed an enlarged lacrimal gland with increased vascularity. The presence of dacryoadenitis, which was unresponsive to initial conservative management with oral antibiotics and warm compresses, along with positive antinuclear antibodies, prompted further investigation. Dacryoadenitis or orbital inflammation is a common presentation in systemic conditions such as Sjogren's syndrome, systemic lupus erythematosus, sarcoidosis, or granulomatosis with polyangiitis. However, it can also be a rare initial symptom in a patient with CREST syndrome. To our knowledge, this is only the second case in the literature of dacryoadenitis in the context of calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia (CREST) syndrome. This case highlights the significance of serological markers and peripheral clinical presentations in individuals with chronic orbital inflammation, emphasizing the importance of considering further systemic associations.

Learning points: Broad spectrum of clinical features: Recognize that although CREST syndrome is primarily characterized by its hallmark features - calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia - it can also present with additional clinical features that may indicate the onset or presence of the syndrome.Diagnostic challenges and differentiation: Diagnostic challenges and differentiation: Bilateral dacryoadenitis can be challenging to diagnose due to its overlapping symptoms with other orbital or systemic conditions. The case report may highlight the importance of differentiating it from conditions like orbital cellulitis, sarcoidosis, or lymphoproliferative disorders. Advanced imaging techniques (like magnetic resonance imaging or computed tomography scans) and careful assessment of clinical history (including systemic symptoms like fever or autoimmune conditions) are crucial for accurate diagnosis.Awareness of atypical presentations and follow-up: The case report underscores the need for tailored management strategies for bilateral dacryoadenitis, from conservative treatments in mild cases to more aggressive therapies in severe ones, while also highlighting the importance of monitoring for complications and being vigilant about atypical presentations, such as those seen in patients with CREST syndrome.