Background/Purpose: IgG4-related disease (IgG4-RD) is a rare multisystem fibro-inflammatory condition, characterized by organ mass lesions, IgG4+ lymphoplasmacytic infiltrate, and storiform fibrosis. To gain better understanding of its prevalence, clinical presentation, and outcomes of treatment, we conducted a retrospective study of patients with IgG4-RD at Cleveland Clinic.

Methods: The Cleveland Clinic pathology database search for IgG4 staining between 2007-2017 found 1,481 results. Of 119 cases positive for tissue IgG4+plasma cells ≥10 and/or IgG4/IgG plasma cells ratio >40%, 57 cases were classified as highly likely (N=28; 49%) if they met at least two of three pathology criteria of lymphoplasmacytic infiltration, fibrosis, and obliterative phlebitis, or probable IgG4-RD (N=29; 51%) if they met only one criteria. Serum IgG4 levels were available in only 15 patients, thus this criterion was not used for classification. Patients’ age, gender, type of treatment, and outcome were retrieved from medical records. Patients were designated as being in “remission” if indicated in the chart and/or symptom and objective findings-free for >6 months, “relapsed”, if symptoms/findings recurred following remission, “active” if no remission was achieved during follow up, and as “unable to determine” if duration of follow-up was <60 days or lost to follow up.

Results: Of 57 patients, 63% were males, age 57.9±14.8 years, 57.9% were white, 26.3% African-American, 1.8% Asian, 1.8% multi-racial and 12.3% unknown. The average follow-up was 2.7 ± 2.2 years. Organs involved were pancreas (26.4%), salivary glands (8.8%), lacrimal glands (7%), biliary ducts (7%), aorta (7%), thyroid (5.3%), orbit (3.5%), lung (3.5%), lymph nodes (1.8%), and others (24.6%). On histopathologic examination, 94.7% had lymphoplasmacytic infiltration, 68.4% fibrosis, 31.6% obliterative phlebitis, and 14 patients (24.6%) met all three criteria. Eosinophilic infiltration was present in 8 patients (14%). Average IgG4+ plasma cell count was 49.7±36.2/hpf. Almost half of the patients (45.6%; N=26) were managed surgically, 21.1% (N=12) medically only, and 24.6% (N=14) received both. Medical treatment included prednisone (45.6%), methotrexate (5.3%), azathioprine (7%); 58.3% of patients took more than one medication, and 5 patients received rituximab (8.8%). Remission was achieved by 52.6% of surgical, 21.1% of medical, and 26.3% of recipients of both treatments. Two of five patients treated with Rituximab achieved remission, during the follow up period of 2.39 (0.33-6.46) years.

Conclusion: In this retrospective study, one of the largest to date, we confirm that IgG4-RD is more common in middle age/elderly men. Only about half of the cases who met the IgG4 staining criteria satisfied the IgG4-RD histopathology criteria. Pathology findings were variable, with only half of the patients having at least two of the characteristics of lymphoplasmacytic infiltration, fibrosis and obliterative phlebitis, and a quarter displaying all three. These results underline the difficulty of making a definitive diagnosis of IgG4-RD. In our cohort, surgical treatment compared to medical treatment appeared to be more successful in achieving remission.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/retrospective-analysis-of-igg4-rd-patient-population-at-the-cleveland-clinic-between-2007-2017/