Biomedical subjects
Publications and source records attributed to B Devulder.
We describe two cases with sarcoidosis affecting upper respiratory tract. This uncommon localization, may occur in patients with previously diagnosed sarcoidosis, but it may be the first or the only manifestation of the disease. A severe upper airway obstruction can occur, necessitating urgent tracheostomy. Systemic corticosteroid therapy is the treatment of choice in most cases.
The diagnostic value of c-ANCA as a specific marker of systemic vasculitis (particularly Wegener's granulomatosis) is well established. The prognostic value of c-ANCA for determining disease activity is controversial. We have prospectively studied in ten patients with systemic vasculitis over a mean period of 34 months (extreme 2-61 months). All patients had c-ANCA at the moment of the diagnosis: four patients had high titer of c-ANCA all over the period study; three clinical and biological exacerbations of the disease was observed without variation of the c-ANCA titer. In four patients c-ANCA disappeared within 6 months after the beginning of the treatment correlated with disease activity. Sometimes a rise of c-ANCA titer was observed with or without disease activity. In one case c-ANCA titer had a serrated evolution. The sensitivity and the specificity of the c-ANCA for disease activity in the ten studied patients were respectively 1 and 0.28. In patients with systemic vasculitis and c-ANCA at the time of the diagnosis, variation in c-ANCA titer alone is of limited prognostic value for predicting disease course.
Dermatomyositis (DM) is characterized by an inflammatory myopathy and typical cutaneous lesions. In a retrospective study, 29 adult cases without overlap syndrome were reviewed. Our aim was to define predictive signs of cancer. Ten (four women, six men) of the 29 patients (35%) had DM associated with malignancy. The diagnosis of cancer was made concomitantly in six cases, more than year after (two cases), more than year prior to (two cases) the occurrence of DM. No extensive diagnosis procedures were necessary for identifying the tumors. A paraneoplasic evolution was certain for any patient, probable for six, improbable for fourth. For the group with malignancy: age was higher (P = 0.01), albumin lower (P = 0.04), C reactive protein higher (P = 0.0061). These parameters seem to have predictive value for the presence of malignancy.
We evaluated the value of serum amyloid P component scintigraphy (123I-SAP) and labial salivary gland biopsy in four patients with apparently localized amyloidosis. One patient had pharyngeal amyloidosis, the second laryngeal amyloidosis, the third retro-ocular amyloidosis and the fourth had carpal tunnel syndrome. Three patients (no 2, no 3, no 4) have abnormal whole body retention of 123I-SAP and positive labial salivary gland biopsy for amyloidosis. Only the first patient had effectively localized amyloidosis and local treatment was sufficient. Melphalan was given to the patients no 2 and no 3, colchicine was given to the patient no 4. Labial salivary gland biopsy and 123I-SAP scintigraphy may give positive argument for the diagnosis of systemic amyloidosis in patients with apparently localized amyloidosis.
We report three cases of primary Sjögren's syndrome in silicotic coal miners. All patients fulfilled the diagnostic criteria for Sjögren's syndrome recently established by the European Community study group. One patient had cryoglobulinemia and polynevritis. Another had Raynaud's phenomenon, arthralgia, purpura and polynevritis. Capillary microscopy was normal in all the three patients. Antinuclear antibodies were detected only in one patient, who had also anti-SSa and anti-SSb antibodies. The prevalence of systemic sclerosis, rheumatoid arthritis and probably systemic lupus erythematosus is significantly higher after longstanding occupational exposure to silica. On the contrary, any case of Sjögren's syndrome was till now described in the course of pulmonary silicosis. The physiopathological mechanisms of these associations are misunderstood.
Polymyositis is characterized by a T-cell-mediated and MHC-I-restricted cytotoxic process, whereas dermatomyositis is a primitively vascular disease with microangiopathy mediated by the complement C5b-9 membranolytic attack complex. We have tried to estimate the frequency of vascular abnormalities in polymyositis as defined by Bohan and Peter. We have retrospectively studied 17 patients with dermatomyositis and 15 patients with polymyositis. Vascular abnormalities have been defined by clinical, capillaroscopic and histologic (muscle biopsy and minor salivary glands biopsy) features. After clinical features, 5/17 dermatomyositis had a Raynaud's phenomenon, against 6/15 polymyositis. Digital necrosis has been observed for 2/17 dermatomyositis and 2/15 polymyositis. In capillaroscopy, 14/17 dermatomyositis had a microangiopathy with or no enlarged capillary loops, against 7/15 polymyositis. None of these polymyositis had enlarged capillary loops. The muscle biopsy showed a predominantly perivascular or perimysial inflammatory infiltrate (vascular process) for 10/16 dermatomyositis against 4/13 polymyositis; a perifascicular atrophy for 3/16 dermatomyositis against 2/13 polymyositis. The histological study of minor salivary glands, showed vascular lesions for 2/11 dermatomyositis and for 1/8 polymyositis. Finally, Bohan and Peter's classification is now inadequate to distinguish between dermatomyositis and polymyositis. Indeed, some dermatomyositis sine dermatitis, may exist and be recognized by their vascular features. To distinguish between dermatomyositis and polymyositis is important, to evaluate the risk of cancer which is more frequent in dermatomyositis.
OBJECTIVE: Several histological studies suggest a vascular involvement in rheumatoid arthritis (RA). We evaluated morphological and functional changes of skin capillaries in patients with RA. METHODS: We studied capillary permeability by nailfold fluorescence videomicroscopy in patients with RA with and without systemic vasculitis to establish the sensitivity, specificity, and the prognostic value of the technique for the diagnosis of systemic rheumatoid vasculitis (SRV). Nine patients with SRV, 22 patients with active RA without signs of vasculitis, and 16 controls were evaluated. RESULTS: No difference in conventional capillaroscopy was observed when we compared patients with SRV and RA. Using fluorescence videomicroscopy after intravenous injection of fluorescein sodium, we observed an increase of transcapillary dye diffusion in 9/9 patients with SRV, in 13/22 patients with RA without vasculitis and in 4/16 control patients. Our results give a sensitivity of 100% in SRV, a specificity of 44%, a positive predictive value of 34% and a negative predictive value of 100%. CONCLUSION: Our present study points out the functional alteration of the capillary wall in patients with RA with or without vasculitis and suggests the usefulness of dynamic fluorescence nailfold capillaroscopy in patients with RA when systemic vasculitis is suspected, not as a diagnostic tool, but because of the high negative predictive value of the technique.
OBJECTIVE: Reports of the detection of amyloidosis by labial salivary gland (LSG) biopsy have been mostly anecdotal. The aim of this study was to assess the value of this method in the diagnosis of amyloidosis. METHODS: LSG biopsy tissues were studied with a combination method using Congo red stain and immunohistologic characterization using an antibody directed against the serum amyloid P (SAP) component. Electron microscopy was performed in all cases. In a prospective study, we evaluated 30 patients with biopsy-proven AA or AL amyloidosis. We compared these patients with a control group of 29 age-matched patients without clinical or biologic evidence of amyloid disease (14 had rheumatoid arthritis and 15 had plasma cell dyscrasia). RESULTS: In 26 of the 30 patients with known systemic amyloidosis, amyloid deposits were identified on LSG biopsy (sensitivity of 86%). In 1 of the remaining patients, amyloid deposits were identified on LSG biopsy and systemic amyloidosis was confirmed by abdominal fat biopsy and 123I-labeled SAP scintigraphy. CONCLUSION: This study emphasizes the high sensitivity of LSG biopsy in the diagnosis of amyloidosis, even in the absence of oral symptoms.
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Cardiac tamponade is very seldom complication of adult onset-Still's disease. We reported 2 cases; it was the first manifestation of the disease in one case.
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Arteriopathies of occupational diseases predominate in the upper limbs and are usually expressed as Raynaud's phenomenon ("white finger"), sometimes complicated by digital gangrene. Hand-arm vibration disease, hypothenar hammer syndrome, exposure to silica even without silicosis and vinyl chloride intoxication are the principal causes of occupational diseases acknowledged as such by the laws. In a personal series of 60 patients with complicated upper limb arteriopathies, 25% had a recognized occupational disease. Since December 1992, Erasmus' syndrome and systemic scleroderma due to silica without silicosis have been accepted in France as being occupational diseases. The therapeutic problems are discussed, notably the surgical indications in hypothenar hammer syndrome, the possible medical treatments of finger necrosis occurring in Erasmus' syndrome and, in particular, the role played by spinal cord neurostimulation.
Sarcoid-like necrotizing granulomatosis is an exceptional condition characterized by lesions of epithelioid granulomatosis and signs of vasculitis. It usually involves the lungs and has a favourable prognosis. We report the case of a young woman with a systemic form of the disease which affected not only the lung, but also the eye and the central nervous system. Under corticosteroid therapy the pulmonary lesions and the biological inflammatory syndrome disappeared, but the brain lesions followed a course of their own despite the addition to steroid treatment of an immunosuppressive therapy. This case highlights the superiority of MRI over CT of the brain in the diagnosis and monitoring of brain lesions: CT became normal after corticosteroid therapy was initiated, where as the neurological signs became worse and the brain lesions progressed on MR images.
The efficacy on plasma lipids, apo A1 and B of a HMG Co A Reductase inhibitor, simvastatin, and a fibrate derivative, gemfibrozil, were compared in 136 hypercholesterolemic patients. The study was randomized, double-blinded and the active drug was given after a 4 week period of placebo. Gemfibrozil (n = 69) was given at 900 mg q.p.m. during the entire study. The primary dose of simvastatin was 10 mg q.p.m. during the first 6-weeks of treatment. At the end of this period, the dose was doubled if the cholesterol level was above 2 g/l (5,16 mmol/l). The same modification was carried out 12 weeks after the beginning active drug treatment with the same criteria. The baselines of total cholesterol were 3.24 +/- 0.69 g/l (8.38 +/- 1.78 mmol/l) 3.21 +/- 0.72 g/l (8.31 +/- 1.87 mmol/l) for patients with simvastatin and those with gemfibrozil respectively. So, at the end of active treatment, 64% of patients in the simvastatin group (n = 67) received 40 mg q.p.m. After 18 weeks of treatment, 89% of patients treated with Simvastatin and 37% with gemfibrozil had more than 20% reduction in LDL cholesterol. Simvastatin was more efficient on total cholesterol, LDL-C and apo B. The increase of HDL-C is similar in both groups of patients. In contrast, the level of triglycerides was further decreased by gemfibrozil. The tolerance was good in the two groups of patients and no difference in the frequency of side effects was observed.
We describe a patient with polyarteritis nodosa who developed a large retroperitoneal hemorrhage from a ruptured suprarenal capsule artery aneurysm. Platinum coils were embolized selectively during arteriography and successfully controlled the aneurysmal bleeding. The vasculitis was controlled ten weeks later but our patient developed anemia and computerized tomography scan revealed a large retrogastric hemorrhage. A further arteriogram confirmed the presence of a ruptured gastric coronary artery aneurysm. A second embolization was performed with complete success. Selective embolization of small arteries with coils should be considered a good alternative to surgery in patients with polyarteritis nodosa and hemorrhage from a ruptured aneurysm; arteriography may be considered a potent diagnostic and therapeutic tool in the management of the treatment of polyarteritis nodosa.
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