Retrospective single-centre analysis of IgG4-related disease patient population and treatment outcomes between 2007 and 2017

Abstract

Objective The aim was to gain a better understanding of the prevalence, organ involvement, clinical characteristics and long-term outcomes of medical and surgical treatments of IgG4-related disease (IgG4-RD). Methods Query of the Cleveland Clinic pathology database for IgG4 plasma cell staining between 2007 and 2017 yielded 1481 results, of which 57 cases were identified as highly likely ( n = 28; 49%) or probable ( n = 29; 51%) IgG4-RD by histopathological criteria and IgG4 serum concentrations. Patient demographics, type of treatment and outcomes were retrieved from medical records. Patients were designated as being in remission if indicated in the chart and/or symptom- and objective finding-free for >6 months, relapsed if symptoms/findings recurred after remission, active if no remission was achieved during follow-up, and as unable to determine if the duration of follow-up was <60 days or they were lost to follow-up. Results Of all patients who met the IgG4 staining criteria ( n = 119), half ( n = 57) satisfied the IgG4-RD histopathological criteria; 63% were males, age 57.9 ± 14.8 years. The average follow-up was 2.7 ± 2.2 years. The pancreas was the most affected organ in our cohort (26.4%). Almost half of the patients (45.6%; n = 26) were managed surgically, 21.1% ( n = 12) medically, and 24.6% ( n = 14) received both types of treatment. Medical treatment included prednisone (45.6%), MTX (5.3%), AZA (7%) and rituximab (8.8%). Remission was achieved by 77% of patients receiving surgical, 67% receiving medical and 72% receiving both treatments. Conclusion A histological diagnosis of IgG4-RD could be made in half of the patients who had IgG4 + plasma cells ≥10/high-power field or IgG4 + /IgG + ratio >40%. In our cohort, surgical treatment compared with medical treatment had a higher proportion of remission according to our outcome classification.

Two cases of immunoglobulin G4 (IgG4)-related hypophysitis diagnosed without pituitary biopsy

“…16,17 MTX is an immunosuppressant used widely due to its high effectiveness, minimal side effects, and low price. 18 In our male patient, multiple organs were involved and MTX was given. Diagnosis of IgG4-RH is difficult due to lack of specific clinical manifestations; 30% of hypophysitis and 4% of hypopituitarism is caused by IgG4-RH.…”

“…Pituitary MRI showed thickening of the upper part of pituitary stalk and loss of the posterior pituitary signal on T1-weighted MRI (Figure 2). Chest CT revealed multiple tiny pulmonary nodules in both lungs, which possess no metabolic activity on 18 F-FDG positron emission tomography (PET)-CT. No other abnormality was shown on the PET-CT.…”

Tumour of the orbit and pterygopalatine fossa: delayed recognition of possible IgG4-related disease

“…Tissue IgG4 numbers and IgG4:IgG ratios are secondary. The guidelines proposed in this statement do not replace careful clinical-pathological correlation and sound clinical assessment [ 7 , 8 ].…”

“…Another alternative is combination therapy. A combination of Mycophenolate mofetil with azathioprine and methotrexate was shown to be effective in about 50% of the cases [ 8 , 17 , 18 ].…”

“…A consensus statement on the pathology contains the scheme based on characteristic histological features such as: dense lymphoplasmacytic infiltrate; fibrosis – storiform in character – and obliterative phlebitis. Depending on the number of pathology features and numbers of IgG4 + plasma cells, the diagnosis of IgG4-RD is histologically highly suggestive or probable [ 7 , 8 ].…”