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X Puéchal

Publications and source records attributed to X Puéchal.

At least 19 recordsLinked to original sources

Thromboangiitis obliterans or Buerger's disease: challenges for the rheumatologist.

Thromboangiitis obliterans (Buerger's disease)--a type of vasculitis in young, mostly male subjects--remains strangely linked to smoking, which determines its occurrence, progression and prognosis by currently unknown mechanisms. It affects the small and medium-sized arteries and veins of the limbs. Despite the usual absence of systemic signs and symptoms, initially intermittent arthritis, even if not observed in all cases, confer on this disease the status of a systemic vasculitis. Diagnosis requires the elimination of many other diseases. The severity of the disease lies in the need for amputation in more than a quarter of all patients. Complete cessation of smoking remains the cornerstone of therapy. Local care is the second essential element of treatment. Prostacycline analogues can be used to help the patient through critical ischaemia. Blockade with antagonists of cannabinoid or endothelin receptors and the use of gene- or cell-based therapy to induce therapeutic angiogenesis have opened up new possibilities for treatment.

Adult↗

Effects of corticosteroids and immunosuppressors on idiopathic inflammatory myopathy related myocarditis evaluated by magnetic resonance imaging.

BACKGROUND: Cardiac involvement in idiopathic inflammatory myopathy has been recognised as an important prognostic factor, but treatment remains empirical. OBJECTIVE: To investigate the effects of corticosteroids and immunosuppressors on myocarditis in patients with inflammatory myopathies. METHODS: Patients with inflammatory myositis of recent onset who had not received treatment were evaluated for associated myocarditis by magnetic resonance imaging (MRI) and reinvestigated after treatment with high dose corticosteroids and immunosuppressors. RESULTS: Four patients with histologically proven myositis were included. Two patients with polymyositis had cardiac clinical symptoms. Two other patients with dermatomyositis and diffuse cutaneous systemic sclerosis-polymyositis overlap syndrome were asymptomatic. In three cases the usual conventional screening tests were normal. For all patients an area of contrast enhancement and hypokinesia detected by cardiac MRI was markedly reduced after treatment with corticosteroids and immunosuppressors for 6 months. CONCLUSION: Treatment with intravenous methylprednisolone followed by prednisone and immunosuppressive therapy seems to be effective for treating myocardial involvement in patients with idiopathic inflammatory myopathies, either alone or presenting as overlap syndromes. Cardiovascular MRI is a non-invasive technique that may be a powerful tool for diagnosis and monitoring of myocardial inflammation in this setting.

Adrenal Cortex Hormones↗

Rheumatoid arthritis and schizophrenia: a negative association at a dimensional level.

There is wide evidence for a decreased risk of rheumatoid arthritis in patients with schizophrenia. Nevertheless, very few studies have looked at the risk of schizophrenia in a group of patients with rheumatoid arthritis. We prospectively investigated, with the SCL-90R, 220 consecutive outpatients with rheumatoid arthritis and 196 consecutive outpatients with various medical conditions, half of them suffering from psoriatic arthritis (a medical condition close to rheumatoid arthritis). The SCL-90R appears to be a valuable tool to distinguish patients with schizophrenia from the outpatients of our sample, the former having more "paranoid ideation" (p = 0.004) and more "psychoticism" (p < 0.001) than the latter. The "paranoid ideation" dimension was significantly lower (25% decrease) in the sample of patients with rheumatoid arthritis compared to the combined control group (p = 0.005), ratings under the median value being more frequent in the former group (p = 0.025). Confounding factors might not explain this difference according to the regression logistic analysis performed. As patients with rheumatoid arthritis have a lower score of paranoid ideation than controls in our sample, even after controlling for age, gender and severity of the disease, these data represent further evidence for a decreased risk of schizophrenia in individuals with rheumatoid arthritis.

Adult↗

Whipple disease and arthritis.

Whipple disease is a chronic, multisystem, curable, bacterial infection that usually affects middle-aged men and has a wide range of clinical manifestations. The most common symptoms are weight loss and diarrhea, preceded in three quarters of cases by arthritis for a mean of 6 years. In most patients, periodic acid-Schiff staining of proximal small bowel biopsy specimens reveals inclusions within the macrophages, corresponding to bacterial structures. However, patients with various manifestations of the disease may have no gastrointestinal symptoms and negative jejunum biopsy results. Before the onset of gastrointestinal symptoms, a strong index of clinical suspicion is the key to diagnosis. The classic setting is long-term, unexplained, seronegative oligoarthritis or polyarthritis with a palindromic or relapsing course, although chronic destructive polyarthritis and spondyloarthropathy have been repeatedly reported. Identification of the Whipple bacterium, Tropheryma whippelii, has led to the development of polymerase chain reaction as a diagnostic tool in patients in the early stages of the disease or with atypical Whipple disease. This technique can be used to detect the bacterium in many tissues and fluids, including synovial tissue and fluid. The recent cultivation of the Whipple bacillus should lead to the development of serologic tests, further facilitating diagnosis. These recent major advances may show that the infection is more frequent than previously suspected and may expand the clinical spectrum of the disease. It may also allow earlier diagnosis, thereby preventing the development of the severe systemic and sometimes fatal forms of the disease.

Adolescent↗

Carpal tunnel syndrome caused by Mycobacterium szulgai.

We describe 2 cases of infection due to Mycobacterium szulgai revealed by a carpal tunnel syndrome (CTS) that was the only clinical manifestation. Both patients regularly cleaned their fish tank with bare hands. The diagnosis was made by isolation of M. szulgai from synovium. The cause of the CTS was a synovitis. Previous synovectomies were ineffective. Improvement was observed with antibiotic treatment. The only way to diagnose this unusual infection is to perform histology of synovium and to isolate the mycobacteria from synovium culture.

Aged↗

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↗

Occurrence of pulmonary complications during methotrexate therapy in rheumatoid arthritis.

Treatment with methotrexate (MTX) in rheumatoid arthritis (RA) can lead to severe side-effects, especially pulmonary and haematological complications. The aim of this retrospective study was to evaluate, during a 6 yr period, the prevalence and severity of bronchopulmonary side-effects in RA patients treated with MTX. A cohort of 130 RA in-patients (106 women, 24 men) treated with MTX was studied for the occurrence of respiratory adverse events. Adverse bronchopulmonary side-effects were observed in 12 patients (two men, 10 women), with a mean disease duration of 15 yr. Only three patients had previously suffered from pulmonary disease. MTX treatment duration was between 1 month and 4.5 yr. The diagnosis was that of hypersensitivity pneumonitis (HSP) in four cases, non-HSP pneumonitis in five patients with one case of Pneumocystis carinii infection, and bronchitis in three cases. The initial respiratory symptoms were not discriminatory between the different conditions. Risk factors were not identified for the occurrence of HSP. HSP always occurred in the first 5 months of treatment. Two patients with HSP died, and another patient with opportunistic infection underwent tracheostomy. HSP represents a potentially lethal side-effect in RA patients treated with MTX. Improved education of patients and physicians should certainly lead to a reduction of both the prevalence and severity of pulmonary side-effects during MTX therapy in RA.

Adult↗

Peripheral neuropathy with necrotizing vasculitis in rheumatoid arthritis. A clinicopathologic and prognostic study of thirty-two patients.

OBJECTIVE: To examine the clinicopathologic features of the noncompressive neuropathies in rheumatoid arthritis (RA). METHODS: We studied 32 patients with RA and peripheral neuropathy whose nerve and/or muscle biopsy specimens exhibited necrotizing vasculitis. Morphologic analysis of nerve specimens included light and electron microscopy studies and teased fiber preparation. Survival was evaluated, and the prognostic values of clinical, biologic, and pathologic features were assessed by Cox proportional hazards model. A prognostic assessment based on the significant variables was devised to estimate the probability of survival of any individual patient. RESULTS: Epi- and/or perineurial vasculitis was observed with the same frequency in the 17 patients with sensory and motor deficit and the 15 patients with sensory neuropathies and was associated with axonal degeneration of an average of 77.7% of the nerve fibers. The mean followup was 7.2 years, and the overall survival rate at 5 years was 57%. A full prolonged remission of the vasculitis was observed in 53% of the patients; relapse occurred in 25%. The factors correlated with mortality, in decreasing order of significance, were clinical cutaneous vasculitis (P = 0.0003), neuropathy affecting 3 or 4 limbs (P = 0.03), and depressed level of C4 (P < 0.05). The prognostic assessment indicated a wide range of 5-year probabilities of survival, from < 1% to 93%. CONCLUSION: Necrotizing vasculitis is responsible for the different patterns of noncompressive neuropathies in RA, including mononeuritis multiplex and distal symmetric sensory or sensorimotor neuropathy. Cutaneous vasculitis, multifocal neuropathy, and depressed C4 level were the 3 independent variables which best predicted mortality. We propose a prognostic assessment according to these variables, to stratify patients to receive more aggressive or less aggressive therapy.

Adult↗