A 54 y.o. woman with seronegative arthritis presents with eye pain.

What is in the differential for her R eye pain and blurry vision?

 Hint: she has no cell or flare, no iritis, no uveitis and no retinal abnormalities

Hint: she has no cell or flare, no iritis, no uveitis and no retinal abnormalities

Our patient had enlargement of the lacrimal gland thought to be ANCA negative Wegener’s granulomatosis ( now known as granulomatosis with polyangiitis).  She had four episodes of swelling of the lacrimal gland since 2015 all of which responded to steroids. The ct showed enlargement of the gland as well as stranding surrounding it.

 anatomy of the lacrimal system

anatomy of the lacrimal system

The diagnosis of Wegener’s is often made by biopsies of the upper respiratory tract but the yield of these biopsies for making the diagnosis is only 50%.   It often involves the lungs or kidneys and biopsies of those sites more often reveal the diagnosis.  Since 1982, when  ANCAs (anti-neutrophil cytoplasmic antibodies ) were first described this blood test has been used to diagnose Wegener’s.  The C-ANCA or cytoplasmic antibody directed against serine proteinase 3 is most specific. It is thought that tumor necrosis factor causes serine proteinase to be expressed on the surface of neutrophils and these are the targets for ANCA. When serine proteinase combines with ANCA oxygen radicals are created which damage blood vessels.

Eye manifestations occur in about 50% of patients with the disease.  Focal vasculitis will produce  conjunctivitis, episcleritis, uveitis and granulomatous vasculitis of the retina and optic nerve as well as lacrimal gland enlargement. Eye and upper respiratory tract disease, i.e. sinus disease, may occur years before lung and kidney involvement.

 lacrimal gland involvement in Wegener's

lacrimal gland involvement in Wegener's

DIFFERENTIAL OF LACRIMAL GLAND ENLARGEMENT

ACUTE- bacterial infection, mumps, Epstein barr, gonorrhea and staph, tuberculosis

CHRONIC- Sjogrens,  thyroid disease, orbital pseudotumor, Wegener’s, Behcet’s,sickle cell disease, amyloid

TUMORS- adenocystic CA, squamous CA, lymphoma

 

Our patient was treated with antibiotics in the ED which had no effect and was switched to steroids with resolution of her swelling.

Khu J, Freedman. Lacrimal gland enlargement as an early clinical or radiological sign in thyroid orbitopathy. American Journal of Ophthalmology Case Reports  2017. https://doi.org/10.1016/j.ajoc. 2016.10.005

Lanza JT, Ku Y, Lucente RE. Har-El. Wegener’s granulomatosis of the orbit: lacrimal gland involvement as a major sign. Am J Otolaryngol 1995 16(2):119-22.

Carrington CB, Liebow A. Limited forms of angiitis and granulomatosis of tWegener’s type. 1966. Amer J med 41:491-527.

Singer O, Mccune W. Update on maintaenance therapy for granulomatosis with poyangiitis and microscopic polyangiitis.  2017. Current Opinion in Rheumatology 29(3) 348-53.