Sarcoidosis最新文献

We present two patients with known sarcoidosis who developed neurosarcoidosis manifested by paranoid psychosis and clinical diabetes insipidus with hypernatremia. Both had gadolinium enhanced magnetic resonance imaging which demonstrated leptomeningeal and hypothalamic enhancement. Both had elevated protein and a lymphocytosis in their cerebrospinal fluid, which improved after corticosteroid therapy. The patients improved clinically with this therapy as well. We suggest that new onset psychosis in a sarcoid patient, particularly with symptoms of hypothalamic/pituitary involvement, should be evaluated for neurosarcoidosis with an MRI and CSF examination. If the results are consistent with neurosarcoidosis, the patient should be treated promptly with corticosteroids.

In pulmonary sarcoidosis or experimental granuloma formation, interleukin-1 beta (IL-1 beta) or tumor necrosis factor alpha (TNF alpha) seem to play important roles during the inflammatory process. In order to examine whether IL-1 beta or TNF alpha mRNA expression in lung macrophages relates to disease activity or clinical course, ten cases with pulmonary sarcoidosis were divided into two groups: five cases with disease duration of more than 10 years (14.6 +/- 4.4 yrs; group A), and 5 cases with a duration of less than 3 years (1.7 +/- 1.1 yrs; group B). All cases showed both abnormal chest X-rays and elevated serum angiotensin converting enzyme activities. We compared these ten cases with 12 healthy subjects (6 nonsmokers: NS and 6 current smokers: S), and 5 cases with idiopathic pulmonary fibrosis (IPF) as disease control. Lavage macrophages were purified using the rosette forming method followed by plastic adhesion for one hour. Thereafter, RNA was extracted according to the AGPC method and amplified by the reverse transcription-polymerase chain reaction (RT-PCR). The results showed that IL-1 beta mRNA was detected in all samples studied, but TNF alpha mRNA expression was different among the groups: 5/5 (100%) in group A, 1/5 (20%) in group B, 5/5 (100%) in IPF, and 12/12 (100%) in healthy subjects. A constitutive expression was seen in healthy controls. On the other hand, no detection of TNF alpha mRNA was shown in pulmonary sarcoidosis. This fact may relate to a spontaneous regression of inflammation in sarcoidosis, as a substantial number of cases in group B may in time regress spontaneously.

Glucocorticosteroids represent the "drugs of choice" for treatment of sarcoidosis. Steroids can be given by all routes of administration. Daily therapy with oral steroids is most widely applied. Initial therapy should consist of prednisolone 30-60 mg/day or its equivalent. Alternate day therapy can be used during the maintenance phase. Inhaled steroids can also be tried during the maintenance phase for treatment of pulmonary sarcoidosis. Other drugs, which may be effective in sarcoidosis, and have a steroid-sparing capacity, are methotrexate, azathioprine, chlorambucil and cyclophosphamide. Chloroquine can be used for chronic skin lesions and potassium para-aminobenzoate may soften fibrotic lesions and keloids. Duration of treatment varies with the clinical situation; from between 6 and 18 months to lifetime. In principle, continuing signs of disease activity and functional impairment require continuing treatment. Determination of on-going activity may be a difficult task. Symptomatic patients with stage II-III pulmonary sarcoidosis, and many extrapulmonary manifestations of the disease, must be adequately treated. Symptom-free patients with deteriorating lung function and/or biochemical signs of disease activity also require treatment. Steroids are not indicated for pulmonary stage I disease (hilar lymphadenopathy) with or without erythema nodosum unless there are troublesome persistent chest symptoms (cough, pain, pressure symptoms) or arthralgia, oedema and pain of the legs.

The frequency and functional prognosis of some major extrapulmonary manifestations (EPM) are reviewed using the literature and our own data. The influence of EPM on the vital prognosis is studied. Our knowledge of functional and vital prognosis in EPM is scanty.

Borrelia burgdorferi, i.e. the etiologic agent of Lyme disease, has been causatively linked to sarcoidosis. To evaluate the possible role of this spirochete in the pathogenesis of sarcoidosis we tested for the presence of antibodies to B. burgdorferi on serum samples obtained from 21 sarcoid patients living in an Italian mountain area where Lyme borreliosis is endemic. No patient showed antibodies to B. burgdorferi. Our data does not substantiate the hypothesis that sarcoidosis may be a borreliosis.

Ocular manifestations are seen in at least one-fifth of sarcoidosis patients. Characteristic biomicroscopic and photographic features raise the suspicion of ocular sarcoidosis. Systemic sarcoid associations and compatible laboratory findings, together with the result of an eventual conjunctival biopsy further support the diagnosis. The diagnosis of chronic ocular sarcoidosis is difficult if other systemic symptoms and signs have been resolved. In these cases taking a careful history and searching for conjunctival granulomatosis and residues of intraocular granulomas are important.

Myocardial involvement in sarcoidosis occurs in approximately twenty-seven percent of patients and may result in a grim prognosis. The diagnosis is mainly clinical and may be supplemented by echocardiography, thallium imaging, Gallium-67 scanning, and myocardial biopsy. The value of corticosteroid treatment is still not well-established and few patients have benefitted from cardiac transplantation. We present a case with suspected myocardial sarcoidosis and congestive heart failure who had dramatic response to steroid therapy on five weeks' followup.