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Abstract: Purpose: Spondyloarthritis (SpA) encompass different diseases with common characteristics, ankylosing spondylitis (AS) being the most typical. Undifferentiated SpA may evolve into AS. In France, SpA and rheumatoid arthritis could have the same prevalence. AS has a profound impact on the quality of live and function of patients as well as social and economic consequences for the society. Key points: There is a mean delay of five to eight years between onset of symptoms and diagnosis of AS. This is due to the fact that radiographic sacroiliitis is delayed. The purpose of an earlier diagnosis is emphasized by the need for a better management, the new diagnostic method including magnetic resonance imaging and ultrasonography, and by the efficacy of anti-TNF therapy. The current criteria (New-York, Amor, ESSG) are classification but not diagnostic criteria. Their sensitivity is insufficient for an early diagnosis of SpA. Several groups are studying methods to ensure an early diagnosis. The group of Berlin has proposed, for patients suffering inflammatory chronic back pain, an algorithm using clinical, radiological and biological signs with, if necessary, search of HLA-B27 and MRI of sacroiliac joints. But this system is theoretical and the group of Maastricht found it of little effectiveness. Furthermore, it does not take account patients with symptoms beginning out of the spine. Conclusion: We believe that only the follow-up of cohorts constituted of patients with early SpA will enable us to improve our knowledge regarding diagnostic criteria and new tools for early diagnosis, as well as outcome, prognosis and early management of SpA and AS. [Copyright &y& Elsevier]

Introduction Le syndrome de Cogan est une pathologie rare dont la prise en charge reste mal codifiée. L’objectif de l’étude était de déterminer l’efficacité de l’infliximab en comparaison aux DMARDs et la corticothérapie seule. Patients et méthodes Nous avons mené une étude rétrospective française nationale incluant des patients atteints du syndrome de Cogan ( n = 40) et une revue de la littérature des cas ( n = 22) en analysant l’efficacité des DMARDs et des anti-TNFa, en particulier l’infliximab. Les données étaient analysées en considérant les différentes lignes de traitements poolées pour tous les patients. Résultats Nous avons inclus 62 patients (31 femmes) (âge médian de 37 ans [2–76]). Au moment du diagnostic, 61 patients (98 %) avaient des symptômes vestibulo-auditifs, avec une surdité bilatérale dans 41 %. Les signes oculaires étaient présents chez 57 patients (92 %), avec une kératite interstitielle dans le 31 (51 %). Le traitement de première ligne se composait de corticoïdes seuls ( n = 43 ; 70 %) ou associés à d’autres immunosuppresseurs ( n = 18 ; 30 %). Une réponse vestibulo-auditive était noté dans 32/39 (82 %) et 15/19 (79 %) avec corticoïdes seuls et associés à des immunosuppresseurs, une réponse oculaire dans 23/28 (82 %) et 10/14 (71 %) et une réponse systémique dans 13/43 (30 %) et 4/18 (22 %), respectivement ( p = 0,8). Soixante et un patients avaient reçu au total 126 lignes de traitement, consistant en corticoïdes seuls ( n = 51 lignes), avec DMARDs ( n = 65) et l’infliximab ( n = 10). Une réponse vestibulo-auditive était significativement plus fréquente avec l’infliximab que DMARDs ou corticoïdes seuls (80 % contre 39 % et 35 %, respectivement), tandis que les taux de réponse systémique et oculaire étaient similaires. L’infliximab était le seul facteur prédictif d’amélioration vestibulo-auditif ( odd 20,7 [intervalle de confiance à 95 % : 1,65 ; 260], p = 0,019). Conclusion Infliximab pourrait induire une réponse vestibulo-auditive à la différence des DMARDs dans le syndrome de Cogan, mais des études prospectives sont nécessaires. [ABSTRACT FROM AUTHOR]

Introduction: Polymyalgia rheumatica (PMR) can be associated with distal swelling indicating an associated RS3PE syndrome. We report a case of PMR associated with oedema of the lower limbs, which resolved rapidly under glucocorticoid therapy., Case Report: A 85-year-old woman presented with a 4 month history of PMR responding to the 2012 EULAR/ACR classification criteria. Examination of the lower limbs revealed pitting oedema bilaterally up to the knees, with mild erythema and warmth. Hypoalbuminemia (30g/L) was present. There was no cardiac, renal or hepatic cause to explain leg swelling. FDG-PET/CT demonstrated increased metabolism in the periarticular area of shoulders and hips. There was no sign of aortitis or neoplasia. Under treatment with prednisone 10mg/day leg swelling disappeared concomitantly to a weight loss of 8kg within 8days., Conclusion: This case, the first to report leg swelling of inflammatory origin in the context of PMR, could indicate an increased vascular permeability caused by inflammation in the elderly., (Copyright © 2018 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.)

Increased production of TNFα by alveolar macrophages and involvement of TNFα in granuloma formation suggest that this cytokine is involved in the pathophysiology of sarcoidosis. The three available TNFα blocking agents have been tested in sarcoidosis refractory to corticosteroids or immunosuppressive drugs. Data are available from isolated case reports or limited series of patients treated in open label trials with favourable issue with anti-TNFα monoclonal antibodies. Two randomized placebo controlled studies evaluated the efficacy of infliximab in pulmonary and extra-pulmonary sarcoidosis, showing that infliximab improves significantly extra-pulmonary disease. There is no significant difference between infliximab and placebo in the treatment of pulmonary manifestations. Etanercept showed no efficacy for treating ocular sarcoidosis in a controlled trial and for pulmonary disease in an open label trial. Paradoxical cases of proven sarcoidosis have been reported in patients receiving anti-TNFα agents for chronic inflammatory rheumatic diseases. A literature review identified 28 cases, including 16 with etanercept, eight with infliximab and four with adalimumab. Although these cases were mainly reported with etanercept, paradoxical sarcoidosis has been reported with the three available anti-TNFα agents, suggesting a class effect. Changes in the cytokine balance may be involved in these cases of induced sarcoidosis, which must be known by the clinician., (Copyright © 2010 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.)

Acute septic arthritis is a medical emergency both for its diagnosis and its treatment. There are numerous predisposing factors of this condition. Staphylococcus aureus is the most frequent isolated bacteria. In 15% of cases, the clinical picture is not a monoarticular involvement, but rather a polyarticular one. Diagnosis is based on the identification of the micro-organism by joint aspiration and/or blood cultures. These bacteriological samples have to be realized before antibiotics administration. Cultures remain negative in 10 to 20% of true cases of septic arthritis. The antibiotic treatment has to be started when the direct Gram stain examination is positive (50% of cases) or if the suspicion is high but the Gram stain negative. It is frequently necessary to search for an associated endocarditis. It is necessary to remember the possibility of a gonococcal arthritis (tenosynovitis, skin lesions). Joint drainage can be achieved either by closed-needle aspiration or by surgical drainage (hip, destructive lesions on radiographs, no satisfactory response to medical aspirations). Duration of antibiotic treatment varies between 6 and 12 weeks, with the exception of gonococcal arthritis (10 days).

Objectives: The aim of this study was to determine the epidemiologic features of bone and joint tuberculosis in the Paris area from 1980 to 1994., Methods: This retrospective study included 206 cases of confirmed bone and joint tuberculosis (in situ specimen) diagnosed from 1980 to 1994 in 7 hospital units (mainly rheumatology clinics) in Paris and suburban areas., Results: The number of cases increased by a mean 12 per year from 1980 to 1989 then by 17 cases per year from 1990 to 1994. In both 1993 and 1994 there were 21 cases per year. The percentage of immigrant patients was 53% from 1980 to 1989 then reached 74% for 1990-1994, predominantly in subjects from black Africa. Only one patient in the series had human immunodeficiency virus (HIV) infection. A favoring factor was found in 23% of the immigrant and in 45% of the native French patients. Mean age was 37 years in immigrants and 58 years in French patients. Compared with French patients, immigrants had a higher rate of multifocal bone and joint tuberculosis, peripheral osteitis and spondylitus., Conclusion: Our findings suggest that the incidence of bone and joint tuberculosis has increased in Paris in 1993 and 1994. Two populations are especially susceptible: the elderly and/or immunodepressed French population and immigrants coming from endemic zones. The role or HIV infection was minimal in this predominantly rheumatology series.

Cysts of the glenoid labrum are ganglia cysts extending near the glenohumeral joint with frequent clinical signs and symptoms of a compression of the subscapularis nerve. They are detected through MR examination. They are frequently associated to instability of the glenohumeral joint.

Neurologic manifestations are not unusual in multiple myeloma. Conversely meningeal and cerebral involvement have been very rarely reported. We report here on three patients with multiple myeloma and meningeal or cerebral involvement (two of them with autopsy study): one case of cerebellar involvement associated with secondary plasma cell leukemia and two cases of meningeal involvement. We reviewed the characteristics of 20 cases of meningeal involvement with demonstration of plasma cells at cerebrospinal fluid analysis (18 previously reported cases and our two patients). Meningeal involvement occurs in patients with initially stage III multiple myeloma in 85% of cases and is associated with the occurrence of plasma cell leukemia in 20% of cases. The most frequent neurologic signs are: confusion (60%), altered consciousness (25%), gait disorder (25%), cranial nerve palsy (25%). Meningismus is rarely present. Diagnosis is based on cerebrospinal fluid analysis after lumbar puncture which should be made after cranial magnetic resonance imaging. The diagnosis of intra-cranial haemorrhage and infectious meningitis have to be cautiously ruled out. Despite treatments (systemic and/or intrathecal chemotherapy, radiation therapy), prognosis is very poor: mean time of survival after the occurrence of neurologic signs is about 2 months.

Erdheim-Chester disease is an endogenous, non-genetically-determined lipidosis characterized by infiltrates of foamy, lipid-laden histiocytes and by bilateral symmetric foci of sclerosis in appendicular long bones. The clinical spectrum ranges from focal bone lesions to systemic disease with life-threatening visceral involvement. In one third of patients, roentgenograms show focal osteolysis within areas of sclerosis. Authors report a new case of Erdheim-Chester disease documented by two bone biopsies in different sites. Features in their patient included: 1) osteolysis and sclerosis of the long bones of the limbs and maxillas, with CT scan evidence of cortical rupture; 2) on magnetic resonance imaging studies, heterogeneous foci of decreased signal intensity on T1 images and heterogeneous areas of moderately increased signal intensity on T2-weighted images; 3) increased serum osteocalcin levels; 4) laboratory evidence of chronic inflammation with no extraosseous manifestations. The clinical, radiological, and pathological features of Erdheim-Chester disease are different from those of Langerhans cell histiocytosis. However, three cases of patients with both conditions have been reported in the literature, suggesting that there may be links between the two diseases.

This retrospective study evaluated treatment with sulfasalazine (SAS) in a mean dosage of 2.1 g/day in 95 patients with rheumatoid arthritis (RA) who were followed-up for 3 months to 4 years. Mean disease duration was 7 years; 79 patients had previously received at least one disease-modifying drug. Four per cent of patients were lost to follow-up. Mean duration of treatment was 15 months (3 weeks-50 months). Treatment continuation rates were 57% at one year, 40% at two years, and 26% at three years. Reasons for discontinuation of SAS included adverse effects (n = 24), inefficacy (n = 33), and death unrelated to SAS therapy (n = 2). In four patients, SAS was discontinued within three months of the first dose because of a severe adverse effect (diffuse erythematous rash, diffuse bullous rash, hepatitis with jaundice, agranulocytosis). SAS-induced biologic markers for lupus were seen in one patient. Furthermore, 12% of evaluable patients developed antinuclear antibodies during SAS therapy. The SAS treatment continuation rate was higher (p = 0.05) among patients under 40 years of age (n = 18) than among older patients. This difference was due to a correlation between age and tolerance with less SAS-induced side effects in patients under 40 years of age (p = 0.03). The SAS treatment continuation rate was unrelated to the duration of rheumatoid arthritis or number of previous maintenance treatments. This study suggests that rheumatoid arthritis patients under 40 years of age exhibit better tolerance to SAS therapy.

In this retrospective study, survival and prognostic factors were analysed in 65 patients with stage II-III multiple myeloma with osteolytic lesions. Multiple myeloma was diagnosed from 1976 to 1984, and patients were treated with conventional chemotherapy. The response rate to initial chemotherapy was 46%. The median survival time was 31 months. The 10-year survival rate was 10%. Four variables were individually prognostic: response to initial chemotherapy, bone marrow plasma cell percentage, the Durie and Salmon staging system, a biological staging system derived from Durie and Salmon's biological criteria regardless of bone lesions. In the multivariate analysis, only two prognostic variables were retained, namely the response to chemotherapy and the biological staging system. No prognostic value was observed for the extent of osteolytic lesions. This study suggests that, in conventionally treated multiple myeloma, long-term survival has improved compared with the previous decade. It also indicates that the extent of osteolytic lesions has little value for the definition of high-risk myeloma.

Arthritis in mucoviscidosis has been described as aseptic arthritis with the picture of oligo or polyarticular intermittent rheumatism, independent of the pulmonary course of the disease, often accompanied by skin signs, sometimes in the form of vasculitis, and without radiological signs. Chronic forms with the presence of rheumatoid factor and/or radiological signs have also been described. The authors found 4 cases of arthritis (incidence 2%) in a retrospective study of 208 patients with mucoviscidosis. These included one case of typical intermittent rheumatism, one of chronic arthritis of the wrist with positive rheumatoid factor, one case associated with purpura, the course of which was linked to pulmonary secondary infections, and one case of polyarthritis with spinal pain which was difficult to classify. Arthritis in mucoviscidosis appears to be a clinically heterogeneous entity, the pathophysiology of which could involve various immune reactions, secondary to a chronic bacterial stimulus of bronchopulmonary origin.

Retrospective analysis of 63 patients with mucoviscidosis (age: 11 to 21 years), treated with pefloxacine, shows the occurrence of arthropathies ascribed to pefloxacine in 9 patients (age: 9 to 20 years), or 14% of the patients under treatment. The dose of pefloxacine was normal (9 to 16 mg/kg/day) in all cases, except one case of overdose (29 mg/kg/day). Mechanical arthralgias, affect the knees, elbows and wrists, resulting in functional discomfort, and frequently accompanied by mechanical synovial extravasation. They always subside after pefloxacine is discontinued. The role of age is essential as the incidence of arthropathies reaches 45% when pefloxacine is first administered between the ages of 15 and 20 years. Retrospective analysis of 37 patients with mucoviscidosis (age: 2 to 20 years), treated with ofloxacine, failed to show any joint complication. In this study, the best joint tolerance of ofloxacine, compared with pefloxacine is statistically significant. Arthropathies induced by pefloxacine represent an original entity which seems related to the cartilage toxicity of quinolones, observed in animal experiments, during growth.

Human leptospirosis of the classical and severe icterohemorrhagic type, usually due to the L. icterohaemorrhagiae serogroup, is frequent in La Réunion. In a retrospective study conducted between 1980 and 1984 in 249 adult patients, the mortality rate was 13 p. 100. Our data and those found in the literature indicate that the main cause of death is pneumopathy, followed by profuse haemorrhages, arrhythmias and cardiovascular collapse. Acute renal failure is common and often severe; it facilitates gastrointestinal bleeding and is of poor prognosis, particularly in patients with prolonged anuria, a possible cause of lethal hyperkalaemia. Other factors of unfavourable outcome have been demonstrated statistically; they include disturbances of consciousness, hypoprothrombinaemia, epigastric muscle rigidity, hyperleukocytosis, thrombocytopenia, high aspartate aminotransferase levels and chronic alcoholism. At the moment, pulmonary, cardiac and haemorrhagic complications concur with renal failure to darken the prognosis of these severe forms of leptospirosis.