Dacryoadenitis and Diffuse Orbital Inflammation: Unusual First Presentations of Churg-Strauss Syndrome

Abstract

Churg-Strauss syndrome (CSS) is a rare form of vasculitis involving small-to medium-sized blood vessels. CSS typically affects blood vessels of the lungs, gastrointestinal system, and peripheral nerves, but can also involve the heart, skin and kidneys. Here we present two CSS patients presenting with unusual ocular manifestations. Although ophthalmic complications remain relatively uncommon in vasculitides such as Churg-Strauss syndrome, these conditions should be considered in patients presenting with ocular manifestations and concurrent ear, nose and throat symptoms, arthralgia or with positive ANCA and eosinophilia.

Orbital inflammatory disease management

“…A positive p-ANCA test is present in up to 70% of patients with CSS. Although it rarely affects the orbit and periorbital area, CSS can present as diffuse orbital inflammation, panniculitis, myositis, and dacryoadenitis [93,[105][106][107][108].…”

“…IVIG, methotrexate, and cyclophosphamide have also been utilized in addition to corticosteroids to induce remission with good results in isolated studies [93,105,106,110]. Two case reports have described success with methotrexate as maintenance therapy [105,107].…”

Orbital inflammatory disorders: new knowledge, future challenges

“…The orbit is a target for many systemic inflammatory conditions, such as autoimmune thyroid disease, sarcoid, Sjogren, lupus, granulomatosis with polyangiitis (Wegener's), inflammatory bowel disease, rheumatoid arthritis, and IgG4 disease [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17]. Orbital inflammation can also occur in the absence of a distinct autoimmune disorder, in which case it is referred to as 'idiopathic orbital inflammation' [18][19][20][21].…”

Ear, nose, and throat involvement in eosinophilic granulomatosis with polyangiitis

“…2). Sinus and soft-tissue masses or polyps can cause an obstruction or mass effect on adjacent structures, such as the orbits; however, orbital pseudotumor is rarer in EGPA than GPA (23). Rhinitis and sinusitis may be more frequent in ANCA-positive than ANCA-negative patients (52 and 37% versus 25 and 11%), but this is not a reproducible finding across different studies (6Á11, 20Á22).…”