Brain and nerve = Shinkei kenkyū no shinpo
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Immunoglobulin G4-related disease (IgG4-RD) is a novel clinical disease entity characterized by elevated serum IgG4 concentration and tumefaction or tissue infiltration by IgG4-positive plasma cells. IgG4-RD can occur in various organs, including the pancreas, lacrimal gland, salivary gland, thyroid, lung, bile duct, liver, gastrointestinal tract, kidney, prostate, retroperitoneum, arteries, lymph nodes, skin, and breast. ⋯ Recently, a few papers have described the involvement of peripheral nerves in IgG4-RD. Here, we describe the concept of IgG4-RD and highlight the involvement of the central and peripheral nervous systems in IgG4-RD.
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Idiopathic inflammatory myopathies (IIMs) are a heterogeneous group of systemic autoimmune disorders characterized by inflammation of skeletal muscle. In Japan, patients with IIMs usually visit a dermatologist, rheumatologist, or neurologist depending on the main symptom. Because most of the patients with IIMs have muscle weakness as a main symptom, muscle biopsy is usually performed to differentiate these from other non-inflammatory myopathies. ⋯ Therefore, other clinical factors, including rash or clinical complications (malignancy, collagen diseases, or interstitial pneumonitis), have been used along with pathological classification. With the recent discovery of new myositis-specific autoantibodies (MSAs) by rheumatologists and dermatologists, it has been suggested that the presence of a MSA is another important factor for classification. To develop useful methods of classification and to reveal the pathological mechanisms of IIMs, further collaborative studies by dermatologists, rheumatologists, and neurologists are necessary.
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Idiopathic inflammatory myopathies (IIMs) are a group of inflammatory muscle disorders of unknown etiology; these include polymyositis (PM), dermatomyositis (DM), and inclusion body myositis. Extra-muscular manifestations such as dermatitis, arthritis, interstitial lung disease (ILD), cardiomyopathy, and enteropathy are occasional complications in patients with PM/DM. Several myositis-specific autoantibodies (MSAs) have been discovered in IIMs; these can help predict clinical characteristics, response to treatment, and prognosis. ⋯ Immunosuppressive agents are steroid-sparing, serving to mitigate corticosteroid-related side effects, thus making combination therapy an effective treatment option. Preventing the progression of physical dysfunction is of prime importance to patients with PM/DM. Dermatologists, neurologists, and rheumatologists should therefore work together to care for these patients before muscular and extra-muscular involvement develop progressively and irreversibly.
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Sjogren's syndrome is a systemic autoimmune disease characterized by xerophthalmia and xerostomia; it is associated with widespread systemic visceral involvement. A wide variety of neurological complications are characteristic features of Sjogren's syndrome, of which peripheral neuropathy is a major neurological manifestation. Based on the predominant neuropathic symptoms, patients can be considered to have several forms of neuropathies, including sensory ataxic neuropathy, painful sensory neuropathy without sensory ataxia, multiple mononeuropathy, multiple cranial neuropathy, trigeminal neuropathy, autonomic neuropathy, and radiculoneuropathy. ⋯ Differential therapeutic responses to corticosteroids and intravenous immunoglobulin can be seen among the various neuropathic forms. In conclusion, the clinicopathological features of neuropathies associated with Sjogren's syndrome are highly variable. The neuropathy classification is important from a therapeutic point of view.