Orbital Pseudotumor - A Rare Variant of Granulomatosis with Polyangiitis

Case Report

Austin J Clin Immunol. 2015;2(1):1024.

Orbital Pseudotumor - A Rare Variant of Granulomatosis with Polyangiitis

Majai G¹, Tarjan P², Dezso B³ and Zeher M¹*

¹Division of Clinical Immunology, University of Debrecen, Hungary

²Department of Internal Medicine, County Hospital Szolnok, Hungary

³Department of Pathology, University of Debrecen, Hungary

*Corresponding author: Margit Zeher, Division of Clinical Immunology, Faculty of Medicine, University of Debrecen, Móricz Zsigmond Street 22, Debrecen 4032, Hungary

Received: June 18, 2015; Accepted: June 25, 2015; Published: June 26, 2015


Granulomatosis with Polyangiitis (GPA) is an autoimmune small vessel vasculitis with unknown aetiology, characterized by necrotizing granulomatous inflammation affecting the renal, pulmonary, upper airways and ocular systems and is often associated with circulating Anti-Neutrophil Cytoplasmic Antibodies (ANCA) against proteinase 3.

Here we report a presentation of GPA showing orbital involvement (without systemic involvement) with the classical histological triad, and also significant localized tissue eosinophilia which corresponds with the rare eosinophilic form of GPA. This variant of GPA should be differentiated from Eosinophilic Granulomatosis with Polyangiitis (EGPA); and the differential diagnosis between the two entities, and also the treatment of GPA is discussed.

Keywords: Orbital pseudotumor; Eosinophilic variants of GPA; Small vessel vasculitis; Anti-CD20 monoclonal antibody therapy


GPA: Granulomatosis with Polyangiitis; EGPA: Eosinophilic Granulomatosis with Polyangiitis; ENT: Ear, Nose, Throat; ANCA: Anti-Neutrophil Cytoplasmic Antibodies; cANCA: cytoplasmic ANCA; MRI: Magnetic Resonance


Granulomatosis with Polyangiitis (Wegener’s Granulomatosis, GPA) is an autoimmune small vessel vasculitis, characterized by necrotizing granulomatous inflammation of Ear, Nose, Throat (ENT), pulmonary, renal, ocular systems, and is often associated with circulating Anti-Neutrophil Cytoplasmic Antibodies (ANCA) against proteinase 3. Its clinical manifestation can be organ/life threatening. Two different phenotypes can be distinguished in GPA, namely the localized/limited and the systemic form [1].

The patients with limited disease have mostly ENT involvement including sinus involvement, nasal septal perforations, nasal collapse and subglottic stenosis. The presence of lung, renal, nervous system involvement is more frequent in patients with systemic form. It was shown that patients with limited disease are nearly a decade younger at disease onset, with longer disease duration compared to patients with severe disease, moreover, the female population is more frequent. The presence of ANCA occurs in 78% of the limited, and 90% of the systemic forms of GPA [2]. The ophthalmological manifestations are present at the diagnosis in about 34% of GPA patients [3] however a long term study showed that 52% of patients developed ophthalmologic manifestations during the course of the disease [4]. The most common ophthalmological manifestations are conjunctivitis (52.1%), episcleritis (39.3%), orbital inflammatory pseudo-tumour (19. 7%) and blurred vision (15.4%) [3].

Case Report

A 38-year-old woman was admitted to our hospital in April 2012 with unilateral exophthalmos, diplopia, and visual impairment. In April 2010 she was admitted to the neurology department with collapse, blurring vision and sweating. EEG, liquor examinations, routine laboratory test were performed and they were within normal range. The Magnetic Resonance Imaging (MRI) identified left side meningeal thickening. In October and November 2010 she presented oral aphtae and arthralgia. The routine laboratory test, hepatitis serology, chest radiography and abdominal ultrasound showed no infection or malignancy. The immunoserology was also negative. In June 2011 she was admitted to the ophthalmology with left side exophthalmos. The MRI identified left side meningeal thickening, left side mastoiditis, and left nonhomogenous orbital mass. At that time, the repeated autoantibody profile was negative. She was treated with high dose of corticosteroid and azathioprine. After 3 month of treatment a control MRI was done, the previously described orbital mass showed no changes. Thereafter an orbital biopsy was performed, and IgG4 level was determined to exclude IgG4 associated disease, malignancy, or orbital inflammation process. The steroid dose was reduced, after a while suspended, and she continued the azathioprin therapy only. During this time, the orbital mass began to increase and the left side exophthalmos became more pronounced. In January 2012 a new orbital biopsy was performed. Histological analysis revealed vasculitis, granulomas formation and tissue eosinophillia.

At her presentation in our department, a left upper eyelid hyperaemia, swelling and exophthalmos were observed. The overlying skin was mobile, the bony margins were palpable and draining lymph nodes were not enlarged. The ENT and systemic examination did not reveal any abnormality. She had no history of atopy, asthma, sinonasal, respiratory or renal disease.

Ophthalmological examination showed dislocation of the left eyeball, diplopia, myopia and secondary glaucoma. Hertel exophtalmometry was used to examine the ocular protrusion. The examination showed 16 mm on right side, 22mm on left side and the interorbital distance was 106mm.

The complete blood count, renal function, electrolytes, thyroid function test, anti –TPO antibody, urine examination did not revealed abnormality. The immunoserology, including ANCA was negative. The previously performed biopsy was requested by our clinic, and a new histological analysis was performed which showed fibroadipose tissue with inflammatory necrosis in the capillaries, venules and arterioles, associated with epitheloid granulomas formation and tissue eosinophillia (Figure 1).

Citation: Majai G, Tarjan P, Dezso B and Zeher M. Orbital Pseudotumor - A Rare Variant of Granulomatosis with Polyangiitis. Austin J Clin Immunol. 2015;2(1):1024. ISSN : 2381-9138