gms | German Medical Science

Medical history: We report the case of a 48-year-old patient, originally from Turkey, who first presented with sialadenitis and sicca symptoms at 20 years of age. He had no significant previous medical history. After years of non-conclusive tests and unsuccessful therapies, he was referred by the ophthalmologists to our rheumatology department for further clarification of a high IgG level.

Course of illness: After the first episode of sialadenitis at the age of 20, he went on to suffer from chronic recurring sialadenitis and underwent numerous biopsies of the parotid gland, which ruled out lymphoma, sarcoidosis and Sjögren’s Syndrome but no firm diagnosis could be made. At 34 years old, the patient developed intra-orbital soft tissue swelling in the right eye which progressed to the left eye 9 years later. Biopsies of the swelling including a lacrimal gland biopsy in 2001 showed no signs of a malignancy or endocrinological orbitopathy but suggested evidence of a high-grade chronic inflammatory reaction with hyperplastic lymphatic tissue, hence an ex juvantibus treatment was trialed with glucocorticoid therapy as well as azathioprine. There was no adequate response to this treatment; therefore, radiation therapy was also tested. This was also unsuccessful in reducing the swelling.

Diagnosis: He was referred to our rheumatology department in 2014. The original lacrimal gland biopsies from 2001 were reanalyzed specifically for an IgG4-associated disease due to a high serum IgG4 level. This revealed dense lymphoplasmacytic inflammatory infiltrates and an increased number of IgG4-positive plasma cells, which are two typical histological features of the disease. The diagnosis of IgG4-associated disease was finally made 28 years after the onset of symptoms.

Treatment: The patient initially responded well to rituximab therapy but relapsed 3 months before the next planned cycle. The therapy was then escalated with methotrexate and tapering prednisolone doses.

Outcome: This treatment combination brought the disease into remission and the intraorbital swelling completely regressed. The patient has been in remission for the last 3 years under rituximab and methotrexate therapy.