[Glucocorticoid sensitive bilateral leg swelling in an 85-year-old woman presenting with polymyalgia rheumatica: A case report]. / Œdèmes des membres inférieurs cortico-sensibles chez une patiente âgée de 85 ans présentant une pseudopolyarthrite rhizomélique.

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.

Characteristics and survival of 26 patients with paraneoplastic arthritis.

OBJECTIVE: To date, only a few series of patients with paraneoplastic arthritis have been published. The charts of patients with cancer-associated arthritis were collected in order to describe characteristics of this rheumatism. METHODS: A questionnaire was created for this study and validated by experts based on specific criteria of inclusion and exclusion. Histology of neoplasia was included. RESULTS: In all, 16 males and 10 females with a mean (range) age of 57.5 years (28-85) were recruited from 17 nationwide centres in France. Patients presented with symmetric polyarthritis involving wrists and hands (85%) and extra-articular symptoms were frequent (84%). There was no specific biologic or radiographic feature. The mean (range) delay between the diagnosis of rheumatism and neoplasia was 3.6 months (0-21.2). Tumours were usually diagnosed after articular symptoms occurred (88.5%). Twenty patients had a solid cancer, and six a haematological malignancy. Adenocarcinoma of the lungs was the most frequent type of solid cancer (60%). Tumours were diagnosed at an early stage, which may explain the good median survival of 1.21 years (range 0.64-present) with a mean follow-up of 1.9 years (range 0.16-10). The percentage of articular symptoms resolution was significantly higher in patients with solid tumours, as compared to patients with haemopathy (p = 0.007). In cases of tumour relapse, rheumatic symptoms did not recur for 75% of patients. CONCLUSIONS: Underlying neoplasia should be considered in male patients with new onset polyarthritis, smokers, and particularly in patients chronically ill. Additional investigations should then be performed to diagnose cancer at an early stage.

Sarcoidosis occuring during anti-TNF-alpha treatment for inflammatory rheumatic diseases: report of two cases.

Anti-TNF-alpha agents have been tried in cases of refractory sarcoidosis, giving favourable results. Thus, the occurrence of a granulomatous disease in a patient receiving such drug seems paradoxical. We describe 2 patients with inflammatory rheumatic disease, the first with ankylosing spondylitis, the second with rheumatoid arthritis, under anti-TNF-alpha treatment (infliximab and etanercept respectively) who developed non-caseating granulomas of the lungs and lymph nodes consistent with the diagnosis of sarcoidosis. Limited and various similar cases have been reported. It is generally considered that these granulomatous diseases are related to the anti-TNF-alpha agent.

[Diagnosis of early spondyloarthritis]. / Diagnostic précoce des spondylarthropathies.

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.

Late-onset neutropenia after treatment with rituximab for rheumatoid arthritis and other autoimmune diseases: data from the AutoImmunity and Rituximab registry.

OBJECTIVES: To evaluate the prevalence of late-onset neutropenia and its complications in patients treated with rituximab (RTX) for rheumatoid arthritis (RA) and other autoimmune diseases (AIDs) in a prospective registry. METHODS: The AutoImmunity and Rituximab registry is an independent 7-year prospective registry promoted by the French Society of Rheumatology. For each episode of neutropenia, data were validated by the clinician in charge of the patient. RESULTS: Among 2624 patients treated with RTX for refractory AIDs, and at least 1 follow-up visit (a total follow-up of 4179 patient-years in RA and 987 patient-years in AIDs), late-onset neutropenia was observed in 40 patients (25 RA (1.3% of patients with RA, 0.6/100 patient-years), and AIDs in 15 (2.3% of patients with AIDs, 1.5/100 patient-years)). 6 patients (15%) had neutrophils <500/mm(3), 8 (20%) had neutrophils between 500 and 1000/mm(3), and 26 (65%) had neutrophils between 1000 and 1500/mm(3). Neutropenia occurred after a median period of 4.5 (3-6.5) months after the last RTX infusion in patients with RA, and 5 (3-6.5) months in patients with AIDs. 5 patients (12.5%), 4 of them with neutrophils lower than 500/mm(3), developed a non-opportunistic serious infection and required antibiotics and granulocyte colony-stimulating factor injections, with a favourable outcome. After resolution of their RTX-related neutropenia, 19 patients (47.5%) were re-treated, and neutropenia reoccurred in 3 of them. CONCLUSIONS: Late-onset neutropenia might occur after RTX and may result in serious infections. Thus, monitoring of white cell count should be performed after RTX. However, in this large registry of patients with AIDs, the frequency of RTX-induced neutropenia was much lower than that previously reported in patients treated for blood malignancies or AIDs.

Spinal tuberculosis in adults. A study of 103 cases in a developed country, 1980-1994.

Spinal tuberculosis (TB) accounts for about 2% of all cases of TB. New methods of diagnosis such as magnetic resonance imaging (MRI) or percutaneous needle biopsy have emerged. Two distinct patterns of spinal TB can be identified, the classic form, called spondylodiscitis (SPD) in this article, and an increasingly common atypical form characterized by spondylitis without disk involvement (SPwD). We conducted a retrospective study of patients with spinal TB managed in the area of Paris, France, between 1980 and 1994 with the goal of defining the characteristics of spinal TB and comparing SPD to SPwD. The 103 consecutive patients included in our study had TB confirmed by bacteriologic and/or histologic studies of specimens from spinal or paraspinal lesions (93 patients) or from extraspinal skeletal lesions (10 patients). Sixty-eight percent of patients were foreign-born subjects from developing countries. None of our patients was HIV-positive. SPD accounted for 48% of cases and SPwD for 52%. Patients with SPwD were younger and more likely to be foreign-born and to have multiple skeletal TB lesions. Neurologic manifestations were observed in 50% of patients, with no differences between the SPD and SPwD groups. Of the 44 patients investigated by MRI, 6 had normal plain radiographs; MRI was consistently positive and demonstrated epidural involvement in 77% of cases. Bacteriologic and histologic yields were similar for surgical biopsy (n = 16) and for percutaneous needle aspiration and/or biopsy (n = 77). Cultures for Mycobacterium tuberculosis were positive in 83% of patients, and no strains were resistant to rifampin. Median duration of antituberculous chemotherapy was 14 months. Surgical treatment was performed in 24% of patients. There were 2 TB-related deaths. Our data suggest that SPwD may now be the most common pattern of spinal TB in foreign-born subjects in industrialized countries. The reasons for this remain to be elucidated.

Adult Henoch-Schönlein purpura associated with malignancy.

OBJECTIVE: Malignancies are among the well-established causes of vasculitis. We studied the association between adult Henoch-Schonlein purpura (HSP) and malignant neoplasms. METHODS: We retrospectively reviewed 14 cases of adult HSP diagnosed during a 6-year period and found a malignant neoplasm in four. Fifteen reports of adult HSP with malignant disease were identified in the literature. These 19 cases were compared with 158 adults who had HSP but no malignancy and who where reported in the literature. RESULTS: Most (63%) of the malignant neoplasms associated with adult HSP were solid tumors: lung, n = 6; prostate, n = 2; breast, n = 1; renal, n = 1; stomach, n = 1; and small bowel, n = 1. Hematologic malignancies (37%) included non-Hodgkin lymphoma, n = 2; Hodgkin disease, n = 2; IgA multiple myeloma, n = 1; myeloproliferative disease, n = 1; and myelodysplastic syndrome, n = 1. Patients with malignancy were older (median age, 59 years), were more likely to be male (M/F = 8.5), had joint involvement more frequently (95%), and had a lower frequency of prior acute infection than those without malignancy. A typical paraneoplastic course was documented in only two cases. CONCLUSIONS: Epidemiological studies are needed to determine the association between adult HSP and malignancy. In practice, it may be wise to suspect a malignancy in men older than 40 years of age who develop HSP in the absence of a precipitating factor. Pathogenic hypotheses involve tumor antigens or abnormal IgA production leading to immune complex formation.

Magnetic resonance appearance of monoclonal gammopathies of unknown significance and multiple myeloma. The GRI Study Group.

STUDY DESIGN: A prospective multicenter study. OBJECTIVES: To evaluate the use of magnetic resonance imaging, in the differentiation between monoclonal gammopathies of unknown significance and multiple myeloma. SUMMARY OF BACKGROUND DATA: Although multiple myeloma has been studied extensively with magnetic resonance imaging, to the authors' knowledge, no study has evaluated the clinical interest of magnetic resonance imaging in the differentiation between monoclonal gammopathies of unknown significance and multiple myeloma. METHODS: The magnetic resonance examinations of the thoracolumbar spine in 24 patients with newly diagnosed monoclonal gammopathies of unknown significance were compared with those performed in 44 patients with newly diagnosed nontreated multiple myeloma. RESULTS: All findings on magnetic resonance examination performed in patients with monoclonal gammopathies of unknown significance were normal, whereas findings on 38 (86%) of the 44 magnetic resonance examinations performed in patients with multiple myeloma were abnormal. CONCLUSION: Magnetic resonance imaging can be considered as an additional diagnostic tool in differentiating between monoclonal gammopathies of unknown significance and multiple myeloma, which may be helpful when routine criteria are not sufficient. An abnormal finding on magnetic resonance examination in a patient with monoclonal gammopathies of unknown significance should suggest the diagnosis of multiple myeloma after other causes of marrow signal abnormalities are excluded. Magnetic resonance imaging also may be proposed in the long-term follow-up of monoclonal gammopathies of unknown significance when a new biologic or clinical event suggests the diagnosis of malignant monoclonal gammopathy.

[Clinical aspects and prognostic factors of icterohemorrhagic leptospirosis in adults. Apropos of 249 cases in La Reunion]. / Aspects cliniques et facteurs pronostiques des leptospiroses ictéro-hémorragiques de l'adulte. A propos de 249 cas observés à la Réunion.

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.

[Neuromeningeal sites of multiple myeloma: 3 cases and review of the literature]. / Localisations neuroméningées du myélome multiple: trois observations et revue de la littérature.

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.

[Epidemiological aspects of osteoarticular tuberculosis in adults. Retrospective study of 206 cases diagnosed in the Paris area from 1980 to 1994]. / Aspects épidémiologiques de la tuberculose ostéo-articulaire de l'adulte. Etude rétrospective de 206 cas diagnostiqués en région parisienne de 1980 à 1994.

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.

[Erdheim-Chester disease: report of a case, review of the literature and discussion of the relation to Langerhans-cell histiocytosis]. / La maladie d'Erdheim-Chester: description d'un cas, revue de la littérature et discussion des rapports avec les histiocytoses Langerhansiennes.

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.

[TNFα blocking agents and sarcoidosis: an update]. / Agents anti-TNFα et sarcoïdose.

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.

Medical therapy of bone and joint tuberculosis in 1998.

Some measure of agreement and no little debate continue to surround the management of bone and joint tuberculosis. There is a consensus that the first phase of antituberculous chemotherapy should consist of three drugs (isoniazid, rifampin, and pyrazinamide) or four drugs (plus ethambutol) given for two to three months. When neither resistance nor side effects occur, isoniazid and rifampin should be continued as maintenance therapy. Patient compliance is well-recognized as requiring special attention because of its large impact on treatment outcomes. Provided strict patient selection criteria and close medical supervision are used, spinal cord compression can be treated nonsurgically with four antituberculous drugs, immobilization and, in many cases, a glucocorticoid. In spinal tuberculosis without neurological signs, immobilization is not always necessary, except when the cervical spine is involved. The optimal duration of antituberculous chemotherapy required for complete recovery of bone and joint tuberculosis is still debated. Twelve months is the duration currently recommended by most experts. Shorter durations, of six to nine months, have been advocated in adults. A critical analysis of the literature shows that proof is still lacking of the equivalence between six- to nine-month and 12- to 18-month treatments. In particular, trials conducted under the aegis of the Medical Research Council of the United Kingdom failed to resolve this issue because of methodological inadequacies regarding sample size and statistical analysis. The large sample size and long follow-up needed to obtain conclusive data would probably require a multicenter international study.

Polyarthritis with perinuclear antineutrophil cytoplasmic antibody inaugurating microscopic polyangiitis. Report of a case.

Microscopic polyangiitis, a condition recently differentiated from macroscopic periarteritis nodosa, is characterized by small vessel damage, pauciimmune necrotizing glomerulonephritis and presence of perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA). Arthralgia is a common symptom often present early in the disease, and other joint manifestations have also been reported. We report a case with polyarthritis as the first manifestation. Perinuclear ANCA was found in a moderate titer. A renal biopsy done six months after the onset of joint symptoms to investigate rapidly progressive renal failure established the diagnosis. The p-ANCA exhibited antimyeloperoxidase specificity. In the discussion we review the diagnosis of microscopic polyangiitis and of concomitant polyarthritis and p-ANCA production. ANCA is present in some patients with rheumatoid arthritis or systemic lupus erythematosus. It is important to determine the specificity of the ANCA since presence of p-ANCA with antimyeloperoxidase specificity in a patient with polyarthritis is highly suggestive of systemic vasculitis.

Magnetic resonance imaging of the spine in plasma cell dyscrasias. A review.

This review of recent data on the techniques and results of spinal magnetic resonance imaging in plasma cell dyscrasias provides a basis for selecting those patients who are most likely to benefit from this investigation. Sagittal images should be obtained using T1-weighted spin-echo and T2-weighted gradient-echo sequences. Epiduritis is best detected on sagittal or axial images acquired after gadolinium injection using T1-weighted spin-echo or phase-opposed gradient-echo sequences. Among patients with symptomatic multiple myeloma, 80% have abnormal magnetic resonance images of the lower spine due to plasma cell infiltration and this proportion increases with the stage in the Durie and Salmon staging system. Bone marrow signal abnormalities can be focal, diffuse and homogeneous, or diffuse and variegated. Vertebral fractures due to spinal infiltration or osteoporosis are seen in 48% of cases and spinal canal narrowing with impingement of bone tumors or epiduritis on nervous structures in 20%. The response to chemotherapy as evaluated based on conventional criteria is fairly well correlated with changes in magnetic resonance imaging findings. Among asymptomatic multiple myeloma patients with normal roentgenograms, 50% have tumor-related abnormalities on magnetic resonance images of the lower spine, which are associated with an increased likelihood of rapid progression to symptomatic disease. Similarly, one third of patients with an apparently solitary plasmacytoma of bone have evidence of other plasma cell tumors on magnetic resonance images of the lower spine, and this finding is associated with persistence of monoclonal component production after irradiation therapy, which may be of adverse prognostic significance. Patients with monoclonal gammopathies of uncertain significance have no evidence of tumorous lesions on magnetic resonance images of the lower spine.

-Cysts of the glenoid labrum-. / Kystes du bourrelet glénoïdien.

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.