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Biomedical subjects

C J Menkès

Publications and source records attributed to C J Menkès.

At least 19 recordsLinked to original sources

Sacral inflammatory pseudotumor revealed by paraneoplastic syndrome.

There is still debate on whether inflammatory pseudotumor should be considered benign or malignant. This lesion has only been reported twice in bone, apart from cases complicating foreign body reaction to joint replacement arthroplasty. We report here a third case, localized at the sacrum. A 31-year-old man had inflammatory dorsalgia and polyarthralgia without synovitis but with fever, asthenia, and erythema nodosa. Biological tests and X-rays were not informative, but technetium scintigraphy revealed a high level of left sacroiliac tracer binding. Several nonsteroidal anti-inflammatory drugs and sulfasalazine treatment were given over 3 months but ineffective. Pelvic magnetic resonance imaging showed an osteolytic tumor of the sacrum. Biopsy suggested a malignant fibrosarcoma, but complete evaluation after surgical resection demonstrated an inflammatory pseudotumor. All clinical symptoms disappeared within a few days after surgery, which is suggestive of a paraneoplastic syndrome. No relapse has occurred after 4 years.

Adult↗

Management of a patient with sarcoid calcaneitis and dactylitis.

Dactylitis and calcaneitis developed in a patient with untreated sarcoidosis of the skin and lungs. Radiographs showed a defect in the second phalanx of the left middle finger. Radiographs of the feet were normal, but magnetic resonance imaging demonstrated a defect in the left calcaneus. Methylprednisolone therapy (1 g per day for 3 consecutive days) induced a dramatic improvement within 48 hours. Low-dose oral glucocorticoid therapy was given subsequently. One year later, a recurrent episode of dactylitis responded promptly to the same regimen. Maintenance therapy was given, and no further recurrences were noted during the four-year follow-up. Sarcoid bone lesions are uncommon and arise selectively in the small bones of the hands and feet. Involvement of the calcaneus is exceedingly rare, and its treatment is not standardized. In our patient, glucocorticoid therapy combined with methotrexate and hydroxychloroquine were effective in controlling the disease.

Adult↗

[Inducible nitric oxide synthase expression and nitric oxide production by monocytes in systemic sclerosis].

We investigated nitric oxide (NO) production and inducible NO synthase (iNOS) expression by cultured peripheral blood mononuclear cells (PBMC) in systemic sclerosis (SSc). Eighteen patients with SSc were compared to two control groups: 16 rheumatoid arthritis patients (RA) and 23 mechanical sciatica patients. The sum of nitrites and nitrates was determined by fluorimetry in sera and spectrophotometry in supernatants. Inducible iNOS was detected in cultured PBMC by immunofluorescence, immunoblot and flow cytometry with or without IL-1 beta + TNF alpha, IL-4 or IFN gamma from day 1 to day 5. NO metabolite concentrations in the plasma were lower in SSc (34.3 mumol/l +/- 2.63 SEM) than in RA (48.3 mumol/l +/- 2.2; p < 0.02) and sciatica (43.3 mumol/l +/- 5.24; p < 0.03) patients. iNOS was detected in cultured monocytes in the 3 groups but induction occurred on day 1 in RA, day 2 in sciatica and only on day 3 in SSc, whatever the stimulus. The concentrations of NO metabolites are decreased in SSc patients and the induction of iNOS in PBMC is delayed. Low levels of NO, a vasodilator, may be involved in vasospasm, which is critical in SSc. This may suggest therapeutic implications.

Arthritis, Rheumatoid↗

Reduced incidence and prevalence of atopy in rheumatoid arthritis. Results of a case-control study.

OBJECTIVE: To determine the cumulative incidence and the point prevalence of atopy in patients with rheumatoid arthritis (RA). PATIENTS AND METHODS: A standardized questionnaire was sent to 300 RA patients. Questions concerned previous or present characteristics of atopy (hay fever, asthma and constitutive eczema) and RA. RA patients were matched with genetically unrelated controls (sister- or brother-in-law, neighbour or friend). The same questionnaire (except for questions about RA) was sent to the control subjects. In cases of atopy, patients, controls and the treating physicians were contacted by a physician to check the validity of the responses. RESULTS: Paired responses were obtained in 173 cases. Information about atopy was obtained for 69 other RA patients. The characteristics of RA were similar for patients who responded and those who did not respond. The frequency of atopy was significantly lower in RA patients than in controls, both for cumulative incidence (RA 7.5%, controls 18.8%; P: < 0.01) and point prevalence (RA 3.5%, controls 16.2%; P: < 0.0001). The clinical manifestations of atopy stopped before the onset of RA in eight of the 17 RA patients with an allergic condition, and there was no subsequent relapse. No effect of RA treatment could account for the remission of atopy. CONCLUSION: These data support the concept that atopy protects against the future development of RA and that the two diseases could counterbalance one another.

Arthritis, Rheumatoid↗

Upper cervical spine surgery in rheumatoid arthritis: retrospective study of 30 patients followed for two years or more after Cotrel-Dubousset instrumentation.

PURPOSE: To evaluate the efficacy of upper cervical spine surgery in symptomatic atlantoaxial instability due to rheumatoid arthritis (RA). MATERIAL AND METHODS: Thirty RA patients (29 women and one man) with a mean age of 56 years were studied retrospectively. Symptomatic forward slippage of the atlas on the axis with a synovial pannus surrounding the odontoid and magnetic resonance imaging evidence of spinal cord compression was present in all 30 patients; 18 patients had vertical translocation of the odontoid and 14 had basilar invagination. Surgery, performed between 1991 and 1997, consisted of occipitocervical fusion in 18 patients and atlantoaxial fusion in 12. Cotrel-Dubousset instrumentation was performed in all 30 patients. RESULTS: Mean follow-up was four and a half years. All patients were satisfied with the procedure and exhibited marked functional gains and objective neurological improvement (by one class in the Ranawat scheme). Stable fusion was documented in all 30 patients. CONCLUSION: Cervical instrumentation and bone grafting seems to provide functional and neurological gains in carefully selected RA patients with atlantoaxial instability and spinal cord compression. Long term follow-up suggests that the benefits are sustained and that morbidity is low.

Adult↗

Prophylactic use of alfacalcidol in corticosteroid-induced osteoporosis.

One hundred and forty-five patients suffering from diseases requiring long-term treatment with high doses of corticosteroids (30 mg/day or greater of prednisolone) were recruited to the study. Patients had to be steroid naive on entry to the study (not more than 15 days of treatment with a corticosteroid within the previous 24 months). Patients were randomized to receive either 1 microgram/day alfacalcidol or placebo capsules for 12 months. Bone mineral density (BMD) of the lumbar spine was assessed by dual-photon absorptiometry on entry and after 3, 6 and 12 months' treatment. Safety was monitored by the recording of all adverse events reported by patients and the regular screening of blood samples for hematology and serum biochemistry. Of the 145 patients, 74 were randomized to alfacalcidol and 71 to placebo. The treatment groups were well matched at baseline with no significant differences in demographic, clinical or biochemical parameters. The mean equivalent dose of prednisolone at baseline was 46.6 mg/day and 46.3 mg/day for the alfacalcidol and placebo group respectively. From the 145 patients randomized to treatment, 71 (38 who received alfacalcidol and 33 who received placebo) provided BMD data both at baseline and at 3, 6 and 12 months. The percentage change in BMD after 6 months' treatment was -2.11% in the alfacalcidol group and -4.00% in the placebo group (p = 0.39). After 12 months the percentage change in BMD was +0.39% (CI: -4.28 to 4.81) in the alfacalcidol group and -5.67% (CI: -8.13 to -3.21) in the placebo group, this difference (6.06%, CI: 0.88 to 11.24) being statistically significant (p = 0.02). An intention to treat analysis also showed a significant difference between the two treatment groups in alfacalcidol's favor (3.81%, p = 0.01; CI: 0.92 to 6.70). There was no significant difference between the two treatment groups in the corticosteroid dose at any time point during the study. Serum calcium was measured throughout and there were no significant differences between the two treatment groups at any visit. This study suggests that alfacalcidol can prevent corticosteroid-induced bone loss from the lumbar spine. Long-term use of alfacalcidol was not associated with any significant adverse effects in this diverse group of patients.

Adjuvants, Immunologic↗

Increased frequency of cystic fibrosis deltaF508 mutation in bronchiectasis associated with rheumatoid arthritis.

This study investigated the clinical characteristics and the possible involvement of the cystic fibrosis transmembrane conductance regulator (CFTR) gene in patients with symptomatic diffuse bronchiectasis (DB) associated with rheumatoid arthritis (RA). Twenty-six patients with both RA and DB (group RA+DB) and control groups of 29 consecutive patients with RA but no bronchiectasis (group RA) and 29 patients with symptomatic DB of unknown origin (group DB) were prospectively studied. Among the patients of the RA+DB group, four (15.4%) were heterozygous for the CFTR gene deltaF508 mutation, whereas no deltaF508 mutation was found in patients of the RA and the DB groups (both, p<0.05). This frequency of deltaF508 mutation was also higher than the expected frequency (2.8%) in the general European population (p<0.04). Sweat chloride values and nasal potential differences were normal in three out of four patients carrying the deltaF508 mutation. In the RA+DB group, those with deltaF508 mutation had more frequent chronic sinusitis (p<0.05), a trend toward a more severe pulmonary involvement, and a lower value of nasal potential differences (p<0.01) whereas their rheumatic features had no particularity. In the RA+DB group, patients with adult-onset bronchiectasis (including two with deltaF508 mutation) had a greater reduction in total lung capacity (p<0.05) and lower nasal potential differences (p<0.005) than those with childhood-onset bronchiectasis. This study suggests a possible deleterious effect of the cystic fibrosis transmembrane conductance regulator mutated protein in the airways which may predispose to the development and severity of bronchiectasis in patients suffering from rheumatoid arthritis.

Adult↗

Rheumatic manifestations in patients with thromboangiitis obliterans (Buerger's disease).

OBJECTIVE: To evaluate articular involvement in a large cohort of patients with thromboangiitis obliterans (TAO). METHODS: A retrospective chart review of all patients with TAO admitted to our rheumatology or vascular medicine departments between 1975 and 1995 was undertaken. Patients with a history of osteoarticular involvement were further investigated. This evaluation entailed taking a detailed rheumatologic and musculoskeletal history and physical examination. Laboratory tests and osteoarticular radiographs were obtained. RESULTS: Initial rheumatic manifestations were observed in 11 (12.5%) (7 male/4 female) of 83 (71 male/12 female) patients with TAO. Eight patients presented recurrent episodes of arthralgia, which preceded the diagnosis of TAO by 2 to 13 years (median 10 years). The episodes were monoarticular, acute, transient, and migratory, affected large joints with local inflammatory signs, and were nonerosive. The initial diagnosis often referred to a loco-regional or periarticular problem that tended to recur. There was permanent disappearance of these prodromal rheumatic features with the appearance of ischemic features. Two patients had HLA-B27+ undifferentiated spondyloarthropathies and one presented oligoarthritis. CONCLUSION: TAO should be considered in patients presenting intermittent rheumatism. The key symptoms and signs of the disease should be monitored during the followup period because the first occlusive features may appear more than 10 years later. Thromboangiitis manifestations can thus be similar to systemic vasculitis and connective tissue disease. If the patient is a young adult smoker, particularly a young man with diminished or absent distal pulses, a diagnosis of TAO should be considered.

Adolescent↗

Nonsurgical synovectomy in the treatment of arthropathy in Von Willebrand's Disease.

Von Willebrand's disease is the most common inherited bleeding disorder, with an overall prevalence in the general population of 0.8% to 1.3%. Hemarthrosis occurs mainly in the severest forms of the disease (type III), with a frequency of 3.5% to 11%, and can cause severe arthropathy similar to that seen in hemophilia. We retrospectively reviewed our experience with nonsurgical synovectomy in the treatment of recurrent hemarthrosis with arthropathy in patients with von Willebrand's disease. Four of our six patients had type III disease and the remaining two had type II disease. The age range was 13 to 63 years. The frequency of hemarthrosis prior to synovectomy was one to four per month. One (n = 2) or both (n = 1) knees were treated in 4 cases, one (n = 1) or both (n = 1) ankles in 3 cases and an elbow in one case. We used yttrium 90 in a dose of 5 mCi for one knee, rhenium 186 in a dose of 2 mCi for two ankles and the elbow and osmic acid for two knees and one ankle. Clinical and radiological results were evaluated six months after synovectomy using the World Federation of Hemophilia score. Radiologic lesions remained stable and clinical manifestations improved in every case (p < 0.05). Five patients achieved a complete remission. Safety was satisfactory. The clinical efficacy of synovectomy done, using radiocolloids or osmic acid in arthropathy due to von Willebrand's disease, seems similar to that in hemophilia.

Adolescent↗

Update on lumbar spinal stenosis. Retrospective study of 62 patients and review of the literature.

PURPOSE AND METHODS: Although lumbar spinal stenosis syndrome is extremely common, considerable controversy continues to surround its classification, diagnosis, and treatment. We retrospectively reviewed the medical charts of 62 patients admitted for lumbar spinal stenosis syndrome, and we compared our findings to those in the literature. There were 31 women and 31 men. Mean age was 71.8 years. The most common symptoms were positional radiculopathy (92%) and low back pain (95%). The more suggestive sign of pseudoclaudication was observed in only 75% of cases, in keeping with earlier studies. Neurologic symptoms consisting primarily in mild motor loss were present in 15 patients. RESULTS: The most common physical finding was pain during spinal extension (70%). Adopting a bent forward position during walking, a very suggestive manifestation, was seen in 15 patients. Imaging study findings included extensive changes in the discs and facet joints, disc protrusion, scoliosis, and degenerative spondylolisthesis. Myelography was rarely needed. There are no criteria sets for lumbar spinal stenosis syndrome. The diagnosis rests on clinical grounds, and extensive imaging studies are usually unnecessary. Our patients were treated with repeated corticosteroid injections into the thecal sac or epidural space and/or into the facet joints. Only eight patients required surgery. CONCLUSION: Our study supports the view that most cases of lumbar spinal stenosis can be managed conservatively.

Adrenal Cortex Hormones↗

Nitric oxide as S-nitrosoproteins in rheumatoid arthritis.

OBJECTIVE: Nitric oxide (NO) is a free radical involved in inflammation and immune reactions. The presence of NO is usually assessed by assaying its degradation products, nitrite and nitrate. NO binds to thiol-containing proteins to form S-nitrosoproteins (S-NP). The aim of this study was to investigate the presence of S-NP, together with nitrite and nitrate, in patients with rheumatoid arthritis (RA). METHODS: Forty patients with RA were studied and compared with 24 patients with osteoarthritis (OA) and 21 control subjects. Fourteen patients were treated with 3 consecutive pulses of methylprednisolone for flares of RA. Nitrite was measured by the Griess reaction, and nitrate by a spectrophotometric assay using nitrate reductase. Spectrofluorometry coupled with the inner filter effect was used for the measurement of S-NP. RESULTS: S-NP was detected in all RA samples, both in serum and synovial fluid (SF). Serum and articular S-NP concentrations were correlated (P < 0.03). In RA, nitrite and S-NP levels were higher in SF than in serum; higher SF levels of the 3 compounds were observed in RA than in OA. S-NP levels in RA patients decreased significantly (P < 0.03) after pulse methylprednisolone treatment, in parallel with the clinical improvement. CONCLUSION: S-NP, a biologically active form of NO, was consistently present in RA, with higher concentrations within the arthritic joint. S-NP assays should be added to nitrite and nitrate assays for the evaluation of NO metabolism. S-NP could be a stable storage form of active NO in RA, and its measurement could be useful in evaluating pharmacologic interventions that modulate NO generation.

Arthritis, Rheumatoid↗

[Pancytopenia in rheumatoid arthritis treated with methotrexate].

OBJECTIVES: Six cases of pancytopenia were analyzed retrospectively among 350 patients with rheumatoid arthritis treated with methotrexate. Pancytopenia is an uncommon but severe secondary effect of methotrexate. CASE REPORTS: Five patients were hospitalized for infectious complications or hemorrhage with favorable outcome. One patient died due to septic shock. There were risk factors in all 6 patients: 5 were over 65 years of age, creatinine clearance was under 50 ml/min in 4, hypoalbuminemia was found in 4 and methotrexate was combined with an antiinflammatory drug in 4 and with ranitidine in 2. The pharmacological imputability of methotrexate was probable in 4 of the 6 patients. DISCUSSION: Acute pancytopenia in patients treated with methotrexate can be prevented by recognizing risk factors, regular laboratory tests and supplementation of all patients with folic acid according to protocols to be established.

Aged↗