Difficult-to-treat rhinosinusitis in IgG4-related disease

Abstract

Introduction: IgG4-related disease is a rare, immune disease with hyper-IgG4-gamma-globulinemia and fibro-inflammatory lesions that can occur in any organ. The diagnosis is made on the basis of clinical and histopathological criteria. In otolaryngological practice, the IgG4-related disease should be taken into account in the differential diagnosis of patients with salivary or lacrimal gland enlargement, with eyelid edema and chronic rhinosinusitis. Systemic glucocorticosteroids are the first line of treatment and can be combined with other immunosuppressants or biological treatment. The prognosis is favorable, but delay in diagnosis and therapy can lead to multiple organ failure and premature death. Case study: The paper presents a case report of patient initially treated for allergic rhinitis with asthma and eyelid edema, accompanied by rhinosinusitis, and history of numerous comorbidities (Hashimoto’s disease, parotid tumor, idopathic hepatitis, anemia and albinism). Imaging studies confirmed enlargement of the salivary and lacrimal glands with the upper eyelids edema and rhinosinusitis. The patient was qualified for sinus surgery. Postoperative histopathological examination enabled the diagnosis of IgG4- -related disease. For the next 5 years, the patient received prednisone and azathioprine, then prednisone and methotrexate, inhaled and nasal steroids, under constant specialist care, including otolaryngologist. Eyelid oedema resolved, asthma and allergic rhinitis were well controlled. Despite treatment, sinus symptoms gradually worsened, accompanied by headaches. The mucocele of the left frontal sinus was diagnosed and qualified for sinus reoperation. In the postoperative period, despite intensive treatment, mucocele of the right frontal sinus developed. Finally, another radical surgery on the frontal sinuses was performed (Draf III). The patient also showed other signs of progression of IgG4-related disease, including a twofold increase in serum IgG4 concentration. The lack of full control of the disease, manifested by difficult to treat rhinosinusitis, became the basis for qualification of the patient, now 44 years old, to biological treatment with rituximab.