Other Organs (Central Nervous System, Prostate)
IgG4-related disease (IgG4-RD) is a systemic lymphoproliferative disease characterized by elevated serum IgG4 values and increased numbers of IgG4-producing plasmacytic cells in tissues. Lesions may develop at sites throughout the body. The disease tends
- PDF / 515,854 Bytes
- 5 Pages / 595.28 x 790.87 pts Page_size
- 60 Downloads / 231 Views
17
Yasufumi Masaki, Nozomu Kurose, Hisao Tonami, and Hisanori Umehara
17.1
Central Nervous System Lesions
17.1.1 Hypertrophic Pachymeningitis Hypertrophic pachymeningitis is a refractory inflammatory disease characterized by fibrous thickening of the dura mater. The causes of hypertrophic pachymeningitis include tuberculosis, fungal, and other infections; sarcoidosis; rheumatoid arthritis; vasculitides such as giant cell arteritis and granulomatosis with polyangiitis (formerly Wegener’s); and malignancies. In a substantial percentage of cases, however, the cause cannot be determined and the etiology is regarded as “idiopathic.” In recent years, an increasing number of the cases previously considered idiopathic have been attributed to IgG4-related disease (IgG4-RD).
Y. Masaki (*) • H. Umehara Department of Hematology and Immunology, Kanazawa Medical University, 1-1 Daigaku, Uchinada, Kahoku, Ishikawa 920-0293, Japan e-mail: [email protected]; [email protected] N. Kurose Department of Pathology and Laboratory Medicine, Kanazawa Medical University, Uchinada, Kahoku, Ishikawa, Japan H. Tonami Department of Radiology, Kanazawa Medical University, Uchinada, Kahoku, Ishikawa, Japan
Hypertrophic pachymeningitis can occur in either the intracranial or spinal dura mater, leading to a variety of symptoms according to the site of disease. With involvement of the intracranial dura mater, headaches, cranial nerve dysfunction, optic nerve injury, and cerebellar damage may result. When the spinal dura mater is involved, nerve root and spinal cord compression cause sensory impairment and muscle weakness [1–8] (Figs. 17.1 and 17.2).
17.1.2 Hypophysitis Nonspecific pituitary inflammation includes the long-recognized disease entity known as lymphocytic hypophysitis. Inflammation of the anterior pituitary gland is called lymphocytic adenohypophysitis, while inflammation extending from the posterior lobe to the pituitary stalk is called lymphocytic infundibuloneurohypophysitis. IgG4-RD has now been reported as a cause of lymphocytic hypophysitis leading to pituitary gland dysfunction, affecting either the anterior or posterior lobe of the gland (including the stalk) or both (Fig. 17.2) [9–16]. The frequency of IgG4-RD as a cause of adenohypophysitis and infundibuloneurohypophysitis compared to other causes, such as sarcoidosis or histiocytosis, is not clear at this time. Diverse symptoms and signs can be associated with pituitary gland dysfunction, according to the specific hormone deficiency that results from hypophysitis. The most commonly affected hormones are adrenocorticotropic hormone (ACTH), thyroid-stimulating hormone (TSH), growth hormone (GH), luteinizing hormone (LH), follicle-stimulating hormone
H. Umehara et al. (eds.), IgG4-Related Disease, DOI 10.1007/978-4-431-54228-5_17, © Springer Japan 2014
113
114
Y. Masaki et al.
Fig. 17.1 Images of IgG4-related hypertrophic pachymeningitis. (a) Anterior section, (b) sagittal section of MRI, and (c) l-[methyl-11C]-methionine (MET)—PET coronal section. On M
Data Loading...
