An Orbital Mass Partially Responsive to Steroid

A 68 year old woman presented with a progressively enlarging orbital mass. MRI of her brain and orbits was consistent with an orbital pseudotumor. Although there was some improvement in the patient’s pain and the size of the mass, it did not fully resolve.The patient had a biopsy demonstrate non-caseating granulomatous inflammation. A chest X-ray and CT chest demonstrated bilateral hilar lymphadenopathy. Further examination and interviewing of the patient revealed several months of joint paints and lower extremity nodules. The patient was diagnosed with orbital sarcoidosis and was started on methotrexate by a rheumatologist

Orbital lesion (pseudotumor and dacryoadenitis) is a manifestation of systemic sarcoidosis

Ocular manifestations of systemic sarcoidosis may emerge at any disease stage. Ocular sarcoidosis is typically characterized by uveitic presentations that predominantly involve the choroid of anterior eye segment. However, orbital pseudotumor, dacryoadenitis, and other rare presentations (optic neuritis, dacryocystitis etc.) also occur in sarcoidosis. Threatened vision loss and poor quality of life are the reasons to prescribe systemic steroids in generalized sarcoidosis. Chest CT is required to identify respiratory diseases when sarcoidosis manifests as uveitis or granulomatous orbital inflammation. Multidisciplinary diagnostic and treatment approach to suspected sarcoidosis provides timely verified diagnosis and management strategy. This paper addresses case report of systemic sarcoidosis in a 34-year-old man. The initial manifestation was vision loss and left orbital pseudotumor. The enlarged subclavian lymph node and its biopsy were suspicious of granulomatous inflammation. Chest CT identified disseminated pulmonary lesions and intrathoracic lymphadenopathy that are typical of sarcoidosis. The results of multidisciplinary examinations verified systemic sarcoidosis. Systemic steroids resulted in clinical and radiological improvement and medicinal regression of the disease. Keywords: sarcoidosis, orbital pseudotumor, dacryoadenitis, adenopathy, computed tomography, multidisciplinary approach. For citation: Burylova E.A., Mamaeva L.A., Berdnikova A.S., Fedorova O.K. Orbital lesion (pseudotumor and dacryoadenitis) is a manifestation of systemic sarcoidosis. Russian Journal of Clinical Ophthalmology. 2021;21(1):40–44. DOI: 10.32364/2311-7729-2021-21-1-40-44.

The Efficiency of Pulse Therapy with Glucocorticoids in Inflammatory Orbital Disorders: Case Report

Orbital pseudotumor represents a benign inflammatory disorder of the orbit, with unspecified etiology, whose clinical presentation can mimic the ocular pathology of Basedow disease, namely Graves’ ophthalmopathy, the two of them representing two of the most common orbital conditions. Imagistic methods and laboratory analyses can establish the diagnosis through orbital MRI images specific for the orbital pseudotumor, especially by dosage of the TSH receptor antibodies (TRAb) which will have increased values in Basedow disease with ocular damage. In both situations, the treatment is represented as a first therapeutic line by administration of corticotherapy in high dosage orally or intravenously, with favorably evolution, which is also observable in the case of our patient who was diagnosed with orbital pseudotumor based on MRI images, with associated thyroid dysfunction with negative TRAb, in which the pulse therapy with Solumedrol has determined the improvement of ocular symptoms and signs.

FRI0210 ORBITAL PSEUDOTUMOR AMONG PATIENTS WITH GRANULOMATOSIS WITH POLYANGIITIS – DATA FROM THE POLISH REGISTRY POLVAS

Background:Orbital inflammatory masses have been described as the common manifestation of granulomatosis with polyangiitis (GPA) occuring in 7- 45% of patients.Objectives:Identification and characterization of patients with orbital pseudotumor among Polish patients based on the national vasculitis registry, POLVAS.Methods:Clinical presentation and management of all GPA patients fulfilling ACR criteria or Chapel Hill Consensus Conference definition included to the Polish registry POLVAS who developed orbital masses in the course of GPA were evaluated.Results:Ocular involvement was found in 114 (27%) of 417 GPA patients registered in POLVAS, 34 (8%) developed orbital masses. Mean patients’ age was 47.8 (range from 19-75) yrs., 23 (67%) were women. Forty four per cent of the patients developed tumor at the beginning of the disease, 56% during relapse. Patients’ characteristics on diagnosis of orbital mass: 24 cANCA, 2 pANCA, and 8 ANCA negative, 9% active smokers and 31% past smokers, 29% had localized disease, 21% early systemic and 50% systemic with organ involvement, 29% had other type of ophthalmological involvement before pseudotumor occurred, 88% had active paranasal sinus involvement, 41% lungs, 15% CNS, 15% skin and 6% heart manifestations. Thirty seven per cent of patients had positive nasal swabs cultures, 50% of which were positive for Staphylococcus aureus. In 65%, tumor occurred during steroid therapy (46% had prednisone more than 5mg/d) and 45% on immunosuppressive treatment (19% when treated with AZA, 16% MTX, 6,5% MMF and 3,5% CYC). Due to orbital mass 86.5% were treated with CYC and 13.5% with RTX. Twenty one per cent had complete remission of the pseudotumor, 76% partial remission and in 3% patients there was no response to the treatment; 43% developed visual impairment, 20% suffered from blindness.Conclusion:Orbital inflammatory mass was not common manifestation of GPA among our patients. The mass developed at the beginning or in the course of the disease, even during immunosuppressive treatment. Orbital masses have been resistant to therapeutic interventions and were accompanied by high risk of visual impairment.Disclosure of Interests:Anna Masiak: None declared, Marcin Ziętkiewicz: None declared, Krzysztof Wójcik: None declared, Katarzyna Wawrzycka-Adamczyk: None declared, Radoslaw Jeleniewicz: None declared, Marta Madej: None declared, Joanna Kur-Zalewska: None declared, Katarzyna Jakuszko: None declared, Małgorzata Wisłowska: None declared, Hanna Storoniak: None declared, Michał Komorniczak: None declared, Barbara Bułło-Piontecka: None declared, Iwona Brzosko: None declared, Małgorzata Stasiek: None declared, Eugeniusz Kucharz: None declared, Alicja Dębska-Ślizień: None declared, Maria Majdan Consultant of: Roche, Amgen, Speakers bureau: Roche, Amgen, Jacek Musiał: None declared, Zbigniew Zdrojewski: None declared