DACRYOADENITIS

Signs and Symptoms

Patients with dacryoadenitis may be highly symptomatic or only mildly distracted by their cosmetic appearance, depending upon the underlying etiology and clinical course. By definition, dacryoadenitis represents an inflammatory enlargement of the lacrimal gland, either due to infection or infiltration in response to the presence of systemic disease.1,2

In acute cases, patients will present with unilateral pain or discomfort in the region of the eye and orbit. Focal swelling and tenderness of the temporal aspect of the upper eyelid is characteristic, as is hyperemia and swelling of the superotemporal bulbar conjunctiva overlying the lacrimal gland. In rare cases, mucopurulent discharge may be seen in the involved eye.3 Patient history may include recent or concurrent fever, lymphadenopathy, malaise and/or upper respiratory infection.3,4 In severe instances, the inflamed gland may cause proptotic displacement of the globe, inducing diplopia.1,5

In cases of chronic dacryoadenitis, patients are typically less symptomatic, reporting only a mild “fullness” of the lid and perhaps slight tenderness to the touch. In general, these patients are more concerned about their apparent lid swelling. Seemingly unrelated in the patient’s mind, there may also be secondary dry eye complaints, including burning, scratchiness or a gritty sensation.6 The history most often conveys a gradual onset of the condition over weeks or months.

Chronic cases tend to be bilateral, with nonerythematous inflammation of the lacrimal gland seen upon inspection. A firm, non-tender enlargement of the lacrimal gland may be noted upon lid eversion and palpation. In rare or untreated cases that linger, local ocular effects such as anterior uveitis are possible, complete with the full complement of circumlimbal flush, along with cells and flare in the anterior chamber.

Pathophysiology

The etiology of dacryoadenitis varies widely and depends largely on the presentation. In general, acute cases tend to be infectious in nature, while chronic cases are more indicative of autoimmune disease.2 Sources of infection may be derived from a broad range of microbes, including viruses, bacteria, fungi and parasitic organisms.1,2,7

Viral pathogens appear to be the most common cause of infectious dacryoadenitis; rubulavirus (mumps), Epstein-Barr virus (infectious mononucleosis), herpes simplex, herpes zoster, cytomegalovirus and rarely human immunodeficiency virus have all been implicated as potential causes.2,6-10 Bacterial etiologies of acute dacryoadenitis include the ubiquitous Staphylococcus, Streptococcus and Haemophilus species, but can also involve the less common Neisseria, Moraxella and Pseudomonas.2,6,7 In some cases, these infections have been known to follow ocular trauma.2

Disseminated systemic infection can also manifest as acute dacryoadenitis; these cases are somewhat rare, however, and may be complicated by their potential to present in a bilateral fashion. Implicated organisms include Chlamydia, Brucella, Mycobacterium (tuberculosis), Treponema (syphilis) and Borrelia (Lyme disease).6,7,10-12

In cases of chronic dacryoadenitis, the underlying cause is typically an infiltrative autoimmune disease, such as sarcoidosis, granulomatosis with polyangiitis (formerly known as Wegener’s granulomatosis), thyroid disease, Sjögren’s syndrome, inflammatory bowel disease or Immunoglobulin G4-related disease (IgG4-RD).1,3,13-17 Accumulation of granulomatous tissue, lymphocytes, plasma cells and edema in the lacrimal glands may be seen in these various disorders, leading to the clinical diagnosis.

Management

The initial step in any case of suspected dacryoadenitis is careful consideration of the differential diagnosis, in an attempt to establish an underlying etiology. Dacryoadenitis needs first to be differentiated from other infiltrative disorders of the orbit, such as idiopathic orbital inflammatory disease (also known as orbital inflammatory pseudotumor) and malignant neoplasia of the lacrimal gland or orbital tissue. The preferred technique for such testing involves T1-weighted enhanced magnetic resonance imaging (MRI), both with and without contrast, of the involved orbit.18 Beyond orbital imaging, surgical biopsy is often helpful in further differentiating these presentations.19 Fine-needle aspiration cytology is an alternative technique for identifying the underlying cause in chronic inflammation of the ocular adnexa.20

In cases of acute infectious dacryoadenitis, treatment will depend upon the specific organism. Culture and staining of any discharge may help to identify bacterial and/or fungal pathogens, while hematology, serology and additional radiographic studies are indicated in suspected cases of systemic infection (e.g., syphilis, tuberculosis, etc.). Specific tests will depend on patient demographics, history and other presenting signs, and may include such investigations as a complete blood count (CBC) with platelets and differential, fluorescent treponemal antibody absorption test (FTA-Abs), rapid plasma reagin (RPR), purified protein derivative (PPD), Lyme titer and chest X-ray.

Viral etiologies such as mumps or Epstein-Barr virus are often self-limiting, and warrant no specific action other than relative isolation, rest and supportive therapy; this may involve cold compresses to the involved area and oral nonsteroidal anti-inflammatory agents to mitigate inflammation and discomfort (e.g., naproxen sodium 250mg to 500mg PO BID). For cases of bacterial infection, a course of broad-spectrum antibiotics is warranted; common choices include cephalexin 500mg QID or amoxicillin 250mg to 500mg PO TID. In cases of systemic infection such as syphilis, chlamydia, tuberculosis or Lyme disease, it is recommended that the patient be comanaged with an infectious disease specialist.

Cases of chronic dacryoadenitis may warrant more extensive testing, particularly in those cases where no established diagnosis exists at the time of presentation. Testing for these patients should include such hematologic and serologic investigations as a CBC with platelets and differential, Westergren erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), angiotensin converting enzyme (ACE), human leukocyte antigen typing (HLA-B27), antinuclear antibody (ANA) and antineutrophil cytoplasmic antibodies (ANCA). A thyroid panel—encompassing thyroid stimulating hormone (TSH), T4, Free T4 and Free T3—may be ordered if additional signs and symptoms suggest such an etiology.

Likewise, testing for Sjögren’s syndrome may be performed in cases that are suggestive of that particular disease; historically, this has consisted of non-specific serologic testing for inflammation in addition to specific antinuclear antibodies, most notably anti-Ro and anti-La.21 Recently, however, a new point-of-care test for Sjögren’s became available in the United States. Called Sjö (Nicox), it uses blood sampled from a simple finger stick rather than venipuncture, which is collected on a paper card; the dried blood is evaluated by a central laboratory for a total of seven biomarkers, including ANA, anti-Ro, anti-La, rheumatoid factor, salivary gland protein-1, carbonic anhydrase-6 and parotid secretory protein.22

Treatment for chronic dacryoadenitis typically involves a course of systemic corticosteroids with a slow taper, especially when associated with conditions like sarcoidosis, granulomatosis with polyangiitis or IgG4-RD. Of course, therapy is dependent upon the unique disease and its relative severity.

It is important to remember that chronic dacryoadenitis is merely a sign of a more widespread condition, which is often best managed by a rheumatologist, endocrinologist or infectious disease specialist.

Treatment of associated ocular inflammation such as anterior uveitis is best accomplished through the use of a topical cycloplegic agent and topical corticosteroids, dosed consistently with the level of inflammation.

Clinical Pearls

The diagnosis of dacryoadenitis is made principally by symptomatology and observation.

There are myriad conditions (thyroid orbitopathy, myositis, orbital cellulitis and tumors of the orbit) that can induce inflammation and injection of the conjunctiva, produce proptosis and or limit ocular motility. All deserve consideration at the onset.

Other entities that mimic dacryoadenitis are prolapsed orbital fat (dermolipoma) and dislocation of a healthy lacrimal gland.

Untreated dacryoadenitis can progress to involve collateral tissue, resulting in preseptal cellulitis, orbital cellulitis and/or orbital mucocele.

Pleomorphic adenoma represents 25% of all lacrimal gland mass lesions occurring most commonly between the second and fifth decades of life.1 Adenoid cystic carcinoma is another common lacrimal gland tumor.1

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16. Lee FJ, Varikatt W, Kairaitis K, et al. IgG4-related dacryoadenitis. Ophthalmology. 2010;117(2):398.e3-4.

17. Koizumi S, Kamisawa T, Kuruma S, et al. Clinical features of IgG4-related dacryoadenitis. Graefes Arch Clin Exp Ophthalmol. 2014;252(3):491-7.

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20. Dhaliwal U, Arora VK, Singh N et al. Clinical and cytopathologic correlation in chronic inflammations of the orbit and ocular adnexa: a review of 55 cases. Orbit 2004; 23(4):219-25.

21. Busamia B, Gonzales-Moles MA, Mazzeo M, et al. Assessing the determination of salivary electrolytes and anti-Ro and anti-La antibodies for the diagnosis of Sjögren’s syndrome (SS). Med Oral Patol Oral Cir Bucal. 2010;15(3):e437-40.

22. Shen L, Suresh L, Lindemann M, et al. Novel autoantibodies in Sjogren’s syndrome. Clin Immunol. 2012;145(3):251-5.