Rieko Murakami

Validation of the comprehensive diagnostic criteria for IgG4-related disease in a SMART registry.

1 Division of Rheumatology and Clinical Immunology, Sapporo Medical University School of Medicine, Sapporo, Hokkaido, Japan, 2 Department of Internal Medicine, Otaru General Hospital, Japan, 3 Department of Otolaryngology, Sapporo Medical University School of Medicine, Sapporo, Hokkaido, Japan, 4 Department of Internal Medicine and Rheumatology, JR Sapporo Hospital, Japan, 5 Department of Gastroenterology, Kushiro City General Hospital, Kushiro, Hokkaido, Japan, and 6 Division of Rheumatology and Clinical Immunology, Department of Medicine, Sapporo City General Hospital,

Predicting therapeutic response in IgG4-related disease based on cluster analysis

Abstract To bring the clinical practice of immunoglobulin (Ig)G4-related disease (IgG4-RD) close to personalized medicine, we classified the patient groups and clarified the therapeutic responses of each group. A total of 147 patients enrolled in our registry were classified into four groups by cluster analysis with the software. The therapeutic responses and prognosis of each group were examined. The cluster analysis classified the subjects into four groups: Cluster 1, patients who presented with prominent hypergammaglobulinemia, elevated levels of serum IgG4, and hypocomplementemia; Cluster 2, patients who presented with eosinophilia, elevated concentrations of serum IgG, IgG4, and IgE, and in whom CRP tended to be positive; Cluster 3, patients with younger onset and serum levels of IgG, IgG4, and IgE and peripheral eosinophil counts lower than the other clusters; and Cluster 4, patients with elder onset and low peripheral eosinophil counts. The amounts of glucocorticoid for maintenance treatment were from 5 to 7 mg/d in all groups, but the amounts were significantly greater in Cluster 1 (patients with hypergammaglobulinemia, elevated levels of serum IgG4, and hypocomplementemia) than in Cluster 4 (elder onset patients, relatively low concentrations of peripheral eosinophils). With regard to the use of immunosuppressants and the relapse rate, there were high frequencies in Cluster 1 and Cluster 3 (younger onset patients who presented with mild elevations of serum IgG and IgG4). On the other hand, Cluster 4 showed a low rate of relapse and often could discontinue steroids. The present results suggest that personalized medicine could be provided in IgG4-RD by classifying patients based on their clinical features.

Other Iatrogenic Immunodeficiency-associated Lymphoproliferative Disorder Presenting as Primary Bone Lymphoma in a Patient with Rheumatoid Arthritis.

Primary bone lymphoma (PBL) is a rare disorder. We herein present a case of other iatrogenic immunodeficiency-associated lymphoproliferative disorder (OIIA-LPD) presenting as PBL. A 63-year-old woman was diagnosed with rheumatoid arthritis and had been treated with methotrexate for seven years. Two months before admission, she suffered from pain in the limbs. Magnetic resonance imaging revealed multiple irregular lesions in the bones of the limbs, which showed an uptake of (18)F-FDG on positron emission tomography. A biopsy of the right radius revealed diffuse large B-cell lymphoma, leading to the diagnosis of OIIA-LPD. She received rituximab-containing regimens resulting in a complete response.

A case of Wegener’s granulomatosis with intracranial involvement

We report a case of Wegener’s granulomatosis (WG) associated with intracranial involvement and brain magnetic resonance imaging (MRI) findings. A 44-year-old woman was diagnosed as having WG with involvement of an ear, lungs and kidneys. A brain computed tomography scan showed a space-occupying lesion with a ring-like enhancement in the left temporal lobe. Findings on a brain MRI, different from those characteristic of a brain abscess, supported an intracerebral granulomatous inflammation due to WG. Immunosuppression, which included corticosteroids, was an effective treatment.