PubMed · 14014106
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A BORTHNE. 1962-09-15. [Polymyalgia rheumatica].. https://pubmed.ncbi.nlm.nih.gov/14014106/
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Melkersson-Rosenthal syndrome is a rare disorder characterized by a triad of recurrent orofacial swelling, recurrent paralysis of the facial nerve and lingua plicata. The complete triad only occurs in 25% of MRS cases. Monosymptomatic or oligosymptomatic variants are more frequent; other findings include granulomas in other facial sites, regional lymphadenopathy, fever, psychotic disorders and hyperplastic gingivitis are associated with MRS. This can be a diagnostic challenge. The etiology and pathogenesis of MRS are unknown. Corticosteroids or clofazimine appear the best therapeutic options.
OBJECTIVE: Chronic involvement of the nervous system is relatively rare in sarcoidosis. We describe 7 cases that fulfil Zajicek's criteria for neurosarcoidosis (NS) and propose some modifications to such criteria. MATERIALS AND METHODS: The patients were admitted for various neurological syndromes: 2 cases presented with chronic lymphocytic meningitis, 4 with spinal cord symptoms, one case was initially confused with multiple sclerosis. Serological tests, immunological screening, cerebrospinal fluid (CSF) analysis, bacteriological and viral testing were performed in all patients. Spinal and cerebral MRI, gallium scan, bronchoscopy with biopsy and bronchoalveolar-lavage fluid analysis, high-resolution computed tomography (HRCT) of the chest, biopsy of the lungs, skin, mediastinal lymph-node and meninges, were useful in diagnosing NS. RESULTS AND DISCUSSION: Laboratory tests showed serum inflammatory abnormalities, but were negative for infectious diseases, while CSF showed inflammatory signs in all patients. MRI revealed meningeal enhancement or hypertrophic pachymeningeal lesions in 4 patients, white matter abnormalities and mass lesions in 2 patients, and a spinal mass lesion in 1 patient. Gallium scan, HRCT, bronchoscopy were positive in most cases. Patients were treated with steroid and immunosuppressive therapy, with improvement in six cases. One patient died from infectious complications. CONCLUSION: A definite diagnosis of NS requires demonstration of non-caseating granulomas affecting nervous tissues. In most cases, histological evidence of systemic disease (probable NS) is sufficient in the presence of compatible alterations in the CNS. In our patients the bronchoalveolarlavage fluid analysis, gallium scan, and chest HRCT were important for diagnosis, while serum ACE was always normal and chest radiographs were not suggestive of sarcoidosis.
OBJECTIVES: To evaluate the relationship between disease damage and bone mineral density (BMD) in women with systemic lupus erythematosus (SLE). METHODS: A cross-sectional study was conducted among 307 women with SLE. Patients attended a single clinic visit that included an interview, physical examination, laboratory testing and BMD measurements (hip and/or lumbar spine). Women were stratified by the Systemic Lupus International Collaborating Clinics/American College of Rheumatology cumulative disease damage index (SDI) > or =1 (Damage) vs SDI=0 (No Damage), and prior use of corticosteroids (CS), yielding four groups: (1) Damage/CS(+) (n=138), (2) Damage/CS(-) (n=23), (3) no Damage/CS(-) (n=100), and (4) no Damage/CS(-) (n=46). RESULTS: Mean age at SLE diagnosis was 32.7 +/- 11.8 yr, 24.4% were African American, 65.0% were premenopausal, and mean SDI +/- S.D. was 1.3 +/- 1.8. In the unadjusted and adjusted models controlling for significant univariate risk factors for osteoporosis, the reference group (Group 1) had significantly lower mean BMD T-scores at the hip and lumbar spine than groups having no disease damage (Groups 3 and 4) independent of CS use status. Similar hip and lumbar spine mean BMD T-scores were observed in women with disease damage with and without CS exposure (Groups 1 and 2). CONCLUSIONS: Women with SLE having disease damage and no CS use had BMD T-scores at the hip and lumbar spine similar to those of women with disease damage and prior CS use. These findings suggest an association between disease damage and lower BMD T-scores in women with SLE.