Nihon rinsho. Japanese journal of clinical medicine
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Sarcoidosis is a chronic systemic disease of unknown causes characterized by noncauseating granuloma in various organs. Neurological involvement occurs in 5-6% of patients with sarcoidosis. Most patients with neurosarcoidosis have extraneurologic abnormalities and extraneurologic biopsies usually support the diagnosis, however, nearly half of the patients with neurosarcoidosis present with neurological manifestations without systemic symptoms. ⋯ Elevated serum ACE level is also not a specific diagnosis test. Any part of the CNS can be involved, but there is predilection for hypothalamus, pituitary gland, leptomeninges, and cranial nerves particularly facial nerve. Although corticosteroids are the main stay of therapy, refractory cases can be treated by immunosuppressive and radiation therapy.
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Although hypercalcemia has long been recognized as a complication of sarcoidosis, the incidence of hypercalcemia (> or = 11 mg/dl) in Japan is probably less than 5%. 1 alpha, 25(OH)2D3 is the main cause for hypercalcemia in sarcoidosis and overproduced by sarcoid granulomata. Gamma-interferon produced by activated lymphocytes and macrophages plays a major role in the synthesis of 1 alpha, 25(OH)2D3. ⋯ Treatment of hypercalcemia and hypercalciuria consists of a low calcium diet, adequate hydration, minimization of exposure to sunlight and reducing overproduction of 1 alpha, 25(OH)2D3. Prednisone, 15 to 25 mg/day, is the drug of choice to reduce the overproduction of 1 alpha, 25(OH)2D3.
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Sarcoidosis is a multisystem disorder of unknown thiology. Patients with sarcoidosis present a variety of manifestations. The clinical feature of this disorder depends on age, gender and ethnicity of the patients, duration and activity of the illness, site and extent of organ involvement. ⋯ The diagnosis of sarcoidosis needs a compatible clinical feature and histologic demonstration of noncaseating granulomas. Additionally, other diseases presenting similar clinical pictures and/or pathologic findings should be excluded. In order to obtain a histologic confirmation of the disease and to assess extent, severity and activity of the disease, the further examinations for diagnostic work-up in patients with sarcoidosis are required after establishing clinical and radiological features.
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The frequency of skin involvement of sarcoidosis is 10-30% of all cases, but the prevalence of a particular type of cutaneous lesion varies among races as well as individual cases. Cutaneous involvement is divided into specific and nonspecific categories. ⋯ Despite the clinical importance of scar infiltrates in the diagnosis of sarcoidosis, it is often overlooked because the lesions are usually small and asymptomatic. There is some clinical importance of cutaneous sarcoidosis; the ease in obtaining lesional samples which enables a histological diagnosis of sarcoidosis, and the association of a particular type of cutaneous lesion with other organ involvement.
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In Japan, elderly patients with sarcoidosis, cardiac sarcoidosis and severe pulmonary sarcoidosis patients have been increased. The patients with ocular manifestations without intrathoracic involvement have been also increased and it is, however, difficult to establish the diagnosis and to confirm such patients as sarcoidosis. To detect the histological findings such as epithelioid cell granuloma is gold standard in diagnosis. ⋯ We performed TBLB in 655 patients with clinical features of sarcoidosis. In 125 out of 293 patients with stage 0, 229 out of 272 with stage I, 66 out of 70 with stage II and 19 out of 20 with stage III patients, epithelioid cell granuloma was found. The effort to getting proper tissue samples of suspected sarcoidosis is important.