[Pseudo-autoinflammatory attacks of temporal arteritis]. / Manifestations d'allure auto-inflammatoire d'une maladie de Horton.

INTRODUCTION: Usually, temporal arteritis progresses as a chronic disease. CASE REPORT: The authors report the observation of a 74-year-old woman who presented with two acute flares of temporal arteritis with headache, fever and inflammatory syndrome, which have spontaneously resolved. DISCUSSION: The observations of auto-inflammatory attacks of arteritis disease are rare, but maybe underestimated. The pathophysiology remains unclear.

Regression of cutis calcinosis with diltiazem in adult dermatomyositis.

Calcinosis cutis is common in several connective tissue diseases such as dermatomyositis, scleroderma or lupus erythematous. In dermatomyositis, it is more likely to concern children than adults but it is not exceptional in adults. Several treatments have been used empirically with inconsistent success. We report a case of adult cutis calcinosis associated with dermatomyositis which responded dramatically to treatment with diltiazem.

Sacral insufficiency fractures: an easily overlooked cause of back pain in elderly women.

Sacral insufficiency fractures (SIF) that usually present as nonspecific pelvic or low back pain are often overlooked in the elderly. In a retrospective study conducted in a department of internal medicine, 16 patients with SIF were identified during a 6-year period. All patients were elderly women (mean age of 81 years) who presented with low back or pelvic pain. Radicular pain in the lower limb was common. Ten patients were bedridden. All 16 patients were osteopenic. Plain radiographs of the pelvis were nondiagnostic in 11 patients. Radionuclide bone scan showed a typical H-shaped pattern of increased uptake in 11 patients, and computed tomographic scan confirmed SIF (9/9 patients). With bed rest and analgesics, outcome was favorable in all patients. This study confirms the nonspecific presentation of SIF and the need to maintain a high index of clinical suspicion to make a prompt diagnosis and avoid unnecessary and sometimes invasive procedures.

[Familial Mediterranean fever, clinical and laboratory findings]. / Maladie périodique, symptomatologie clinique et biologique.

Familial Mediterranean fever is the best known of the recurrent hereditary autoinflammatory diseases. It predominantly affects subjects of Mediterranean origin, Sephardic Jews in particular. Its gene, MEFV, is located on chromosome 16 and has autosomal recessive transmission, with incomplete penetration. It codes for a protein called pyrin or marenostrin, which is probably involved in the inflammatory process. In most cases, the first episodes appear before the age of 20 years and very rarely after the age of 40. Episodes usually last a few days, although they may extent over several weeks when localized in joints. Fever, occasionally pseudo-malaria, may accompany various symptoms, the most frequent of which are abdominal, articular, pleural or cutaneous. The abdomen is the classic site of this disease, and acute abdominal flares masquerade as abdominal emergencies. Musculoskeletal involvement is revealed by episodes of inflammation of the joints (more often mono- than oligoarthritis) and muscle pain. The flares are usually brief and totally reversible. Flares of thoracic pain corresponding to pleural inflammation and erysipelas-like skin eruptions have been observed. Acute symptoms disappear between flares, but hepatic splenomegaly, swollen lymph nodes or abnormal fundus of the eye may persist. Laboratory findings are typical of nonspecific inflammation, accompanied by moderate hyperleukocytosis during the flares.

[Familial Mediterranean fever among the autoimmune diseases]. / La maladie périodique au sein des maladies auto-inflammatoires.

During the first attacks of familial Mediterranean fever, each of the disease symptoms can suggest a series of disorders. When the disease is older, the recurrence of symptoms may simulate some systemic diseases, but mainly suggests familial Mediterranean fever, one of a group of hereditary autoinflammatory diseases. Before the gene for familial Mediterranean fever was identified, various sets of criteria were used for diagnosis. The presence of MEFV mutations confirms the diagnosis, but the clinical criteria still determine who should undergo this genetic testing. The genotype-phenotype correlations add a prognostic dimension to the mutations identified. Genotyping can also lead to the diagnosis of the other autoinflammatory diseases, which constitute the basis of the differential diagnosis of familial Mediterranean fever. The hyperimmunoglobulinemia D syndrome (HIDS) is very similar to familial Mediterranean fever in its recessive transmission and abdominal and articular symptoms. It can be distinguished by the European origin of the patients, the presence of cervical lymph nodes and the increased IgD levels. Of the diseases with dominant transmission, the TNF receptor-associated periodic syndromes (TRAPS) are suggested by periorbital edema and migrating inflammatory cellulitis. Muckle and Wells syndrome is revealed by episodes of fever with urticaria and arthralgia, complicated by deafness and amyloidosis. Mutations in the same gene are responsible for two disorders, both appearing in childhood: familial cold urticaria syndrome (FCUS) and chronic infantile neurocutaneous articular syndrome (CINC). The pathogenesis of familial Mediterranean fever is still unclear. Pyrin/marenostrin, the protein produced by the MEFV gene, appears to hae a physiological antiinflammatory effect that inhibits proinflammatory cytokines. Mutation of the gene may eliminate this feedback mechanism and expose the patient to flares from any inflammatory stimulus, even minimal. Amyloid is produced by the serum amyloid A protein (SAA), and its occurrence is influenced by the type of MEFV mutation, but also the genotype of the gene producing SAA.

[From familial Mediterranean fever to amyloidosis]. / De la maladie périodique à l'amylose.

The progression of familial Mediterranean fever is marked by the recurrence, at varying intervals, of acute flares that regress spontaneously. Prognosis, which depends on the occurrence of amyloidosis, has been transformed by colchicine treatment. Incidence of amyloidosis is higher in certain ethnic groups (Jews from North Africa, Turks) and depends on by the specific MEFV mutation. Amyloid is composed of clusters of protein strands identical to the AA protein of secondary amyloidosis and infiltrates the walls of all arterioles except those of the central nervous system. The earliest and most consistent localization is in the kidney, where it develops over several years and in 4 stages--preclinical (latency), proteinuric, nephrotic and uremic--before concluding in end-state renal failure. Before the advent of colchicine, dialysis and transplantation, only renal amyloidosis caused clinical manifestations and lethal complications; any amyloidosis at any other sites remained latent. Prolonged survival with hemodialysis and kidney transplantation now leaves time for manifestation of these other localizations, such as infiltration into the intestines causing malabsorption, or potentially lethal cardiac lesions. Treatment of familial Mediterranean fever is based on the continuous administration of colchicine, which at the average dose of 1 to 2 mg per day can prevent flares or at least reduce their frequency or intensity. Systematic use of colchicine also prevents the onset of amyloidosis, even in the rare cases where it cannot prevent flares. These data fully justify the systematic use of colchicine for continuous prophylactic treatment from diagnosis and even after kidney transplantation, to prevent recurrence of the grafted kidney or extension to other organs. The curative efficacy of colchicine on flares is debatable, although several studies report positive results against progression of early amyloidosis.

[Consequences of replacing abacavir for indinavir in successful antiretroviral treatment of a patient with HIV infection]. / Conséquences de la substitution de l'indinavir par l'abacavir chez un patient infecté par le VIH en succès thérapeutique.

INTRODUCTION: Simplification of combined antiretroviral therapy in HIV-infected patients is possible, but virological success can be compromised by the development or emergence of resistant viruses. CASE: Worsening renal functioning in a patient under successful combination antiretroviral therapy resulted led to the replacement of indinavir by abacavir. Eight weeks later, his viral load rose and he developed a mutant virus resistant to all the nucleoside analogs. DISCUSSION: Our case report illustrates the danger of streamlining combined antiretroviral therapy composed only of nucleoside analogs in patients already successfully treated with nucleoside analogs, by exposing them to the risk of the emergence of a mutant virus.

Pharmacokinetic interactions between NSAIDs (indomethacin or sulindac) and H2-receptor antagonists (cimetidine or ranitidine) in human volunteers.

The reciprocal effects on pharmacokinetic parameters after a single oral dose of the nonsteroidal antiinflammatory drugs (NSAIDs) indomethacin and sulindac and repeated oral doses of the H2-receptor antagonists cimetidine and ranitidine were determined in two groups of nine healthy subjects each (indomethacin and sulindac groups). Administration of NSAIDs increased the AUC and decreased the oral clearance and apparent volume of distribution of the H2-receptor antagonists without modifying their t1/2. Urinary data and observed modifications in ranitidine and cimetidine metabolites seem to justify a greater increase of H2-receptor antagonist bioavailability with indomethacin (p less than 0.05) than with sulindac (NS). The administration of ranitidine significantly reduced the sulindac volume of distribution without modifying its clearance, which caused an increase in the maximum concentration and a decrease in the t1/2 (p less than 0.05). The effects of cimetidine on the two NSAIDs were more intense than the effect of ranitidine: the decrease in sulindac volume of distribution (p less than 0.02) was accompanied by a significant reduction in sulindac clearance (p less than 0.05). AUC and urinary amounts of sulindac's sulfone metabolite were decreased. These results show that NSAIDs increased the bioavailability of H2-receptor antagonists, and that the latter drugs decrease the volume of distribution of NSAIDs. Furthermore, cimetidine modifies the oxidation metabolism of sulindac.

Tuberculosis of the sacroiliac joint: clinical features, outcome, and evaluation of closed needle biopsy in 11 consecutive cases.

Sacroiliac joint (SIJ) involvement has been reported in up to 9.7 percent of patients with skeletal tuberculosis. Lack of awareness of this now uncommon form of infection often leads to diagnostic delay and increased morbidity. Eleven consecutive cases of SIJ tuberculosis are reported; clinical and radiologic features, diagnosis, treatment, and outcome are discussed. Buttock pain was the presenting complaint in all patients. However, radicular pain in the lower back (seven patients) or lower limb (10 patients) was common and in one patient precipitated an unnecessary surgical intervention. SIJ tuberculosis is frequently an isolated phenomenon. Therefore, direct sampling of the SIJ is necessary to establish the diagnosis. The recently described technique of closed needle biopsy of the SIJ was employed in all 11 patients and established the diagnosis in nine of the 11.

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.

Osteoclastoma-like giant cell tumor of the renal pelvis associated with papillary transitional cell carcinoma.

This report documents an uncommon case of osteoclastoma-like giant cell tumor of the renal pelvis associated with papillary transitional cell carcinoma, which resulted in the patient's death. A low-grade transitional papillary carcinoma associated with a prominent osteoclastoma-like giant cell tumor was found in the pelvicalyceal system of the right kidney in a 69-year-old man. The spindle component of the tumor stained for epithelial membrane antigen, indicating an epithelial origin. Positive staining with antibody against p53 protein was observed in both tumoral components. Our results suggest that this osteoclastoma-like giant cell tumor was truly neoplastic and that the two components could have arisen from an initial monoclonal neoplastic proliferation.

Primary Epstein-Barr virus-related non-Hodgkin's lymphoma of the pleural cavity following long-standing tuberculous empyema.

Primary non-Hodgkin's lymphomas of the pleural cavity have been described mostly in Japan. We report a case of high-grade non-Hodgkin's lymphoma (immunoblastic type) of the pleural cavity occurring in a nonimmunocompromised patient 55 years after an artificial pneumothorax was performed for the treatment of pulmonary tuberculosis. Immunohistochemical study revealed a B phenotype (CD20), and an in situ hybridization detected small nuclear RNAs encoded by Epstein-Barr virus in most lymphomatous cells. A link between primary pleural lymphoma and the local long-standing chronic inflammation, inducing a clonal transformation of Epstein-Barr virus-infected immortalized B lymphocytes, is suspected.

[Lumbar post-puncture syndrome]. / Le syndrome post-ponction lombaire.

About 30% of lumbar punctures are complicated by the lumbar puncture syndrome the main symptom of which is a characteristically posture-dependent headache (the so-called "spinal headache"), sometimes accompanied by nausea, vomiting and stiff neck. The syndrome usually begins in the days which follow lumbar puncture and subsides within 10 days. The most common pathogenetic theory is that perforation of the dura mater results in CSF leakage responsible for a fall in intrathecal pressure. Treatment of the lumbar puncture syndrome consists of rest in supine position and copious hydration, usually by the oral route. The epidural blood patch technique is seldom used. Prevention relies entirely on the use of small calibre lumbar puncture needles; keeping the patient lying supine after the puncture is a classical recommendation, but its preventive value has not yet been fully demonstrated.

[Measurement of body temperature in daily practice]. / Mesure de la température corporelle en pratique quotidienne.

Accurate measurement of body temperature is essential in routine medical practice. Development of new measurement methods, and awareness of mercury hazards are leading to the modification of our current practices. The range of body temperature in healthy individuals is 36.8 +/- 0.4 degrees C, but it may be influenced by many factors. Therefore, body temperature should be measured in a standardized fashion, preferably at rest, in the morning on waking. Febrile state is generally defined as a morning rectal temperature above 37.5 degrees C. Body temperature is largely dependent of the site of measurement. Rectal temperature is the highest, and oral temperature measured in the sublingual pocket close to the lingual artery generally the lowest. Tympanic membrane temperature is assumed to best reflect the core temperature. Axillary and cutaneous measures are unreliable. Mercury-in-glass thermometer used to measure rectal temperature still remains the reference in France. However, rectal measurement is inconvenient and associated with a risk of rectal injury and cross-infection. Moreover, mercury-in-glass thermometer results in high mercury pollution. Tendency is to replace it by either electronic thermometer to measure oral temperature, or infrared thermometer for tympanic membrane measure. Although reliable, chemical measures are still underused.

[Uterine localization of periarteritis nodosa disclosed by fever of long duration]. / Localisation utérine d'une périartérite noueuse révélée par une fièvre au long cours.

We report the case of an 81 year-old woman admitted because of declining general health, fever and elevated sedimentation rate. Identification of a pelvic mass on the computed tomographic scan led to perform hysterectomy with a working diagnosis of ovarian malignancy. Histological examination revealed typical lesions of polyarteritis nodosa of uterus and fallopian tube vessels. Diffuse process of the vasculitis was suggested by persistence of fever and elevated erythrocyte sedimentation rate after hysterectomy. Treatment consisting of high-dose corticosteroids and pulses of cyclophosphamide resulted in prompt clinical and biological improvement. We discuss the incidence, the clinical features and the localized or diffuse nature of genital involvement in polyarteritis nodosa and other vasculitis.

[Extra-meningeal meningococcal infection: report of 14 cases]. / Localisations extraméningées des infections à méningocoque : à propos de 14 cas.

INTRODUCTION AND METHOD: Fifty-five patients (17 adults, 38 children) with meningococcal infection were admitted between 1986 and 2002 in a university hospital (500 beds). Fourteen of them (nine adults, five children) presented with an extra-meningeal infection. We compared adults and children presentations. RESULTS: All adults had immunodeficiency. Septic locations were various (three bacteriemia, four pneumoniae, one infected ascitis, one cutaneous abscess). All patients received amoxicillin or third generation cephalosporin. Hospitalisation was prolonged (mean: 47 days). Seven patients required intensive care unit admission, and two of them died. All children (all were less than 36-month-old) presented with fever. Only one was immunodeficient (infected by human immunodeficiency virus). Neisseria meningitidis grew from blood in four, and in the throat for the remaining one. Hospitalisation was of short duration (mean: 4 days) and none of the children required intensive care unit. All the children recovered rapidly with antibiotics. CONCLUSION: Outcome of extra-meningeal infection with N. meningitidis is different in adults and children. Adults present with immunodeficiency, infection is severe and patients present with various clinical features; children have a more homogeneous clinical presentation (fever) and outcome is excellent.

[Febrile form of ankylosing spondylitis]. / Les formes fébriles de la spondylarthrite ankylosante.

INTRODUCTION: Ankylosing spondylitis is a chronic inflammatory disorder that is rarely associated with severe systemic manifestations. EXEGESIS: We report the case of a 29-year-old man presenting with an atypical ankylosing spondylitis. The patient presented with a high spiking fever, a marked weight loss, and an unusual increase of acute phase response markers. Such features are rarely associated with ankylosing spondylitis and the review of the few similar reported cases in the literature showed that this presentation is usually associated with a severe disease course, and an asymmetrical joint involvement that is unresponsive to nonsteroidal anti-inflammatory agents. CONCLUSION: Clinicians should be aware of this unusual presentation of ankylosing spondylitis, which should be included in the list of aetiologies of fever of unknown origin, to avoid useless diagnostic procedures. The severity of the disease course may lead to the consideration of a more aggressive treatment strategy.

[Osteoarticular tuberculosis. Diagnostic contribution of local sampling]. / Tuberculose ostéoarticulaire. Apport diagnostique du prélèvement local.

Bone and joint tuberculosis have recently gained a renewal of interest, especially with the spread of HIV infection which may increase its frequency. Bone and joint locations of tuberculosis are pauci-bacillary often requiring local sampling in order to confirm the diagnosis and to initiate early therapy. From 1983 to 1992 we have studied 19 patients with bone and joint tuberculosis. Seventeen local sampling were performed: 12 biopsies and five abscess punctures. Pathological examination of samples disclosed diagnosis of tuberculosis in eight cases out of 12. Among the remaining four patients, direct smear was positive once, and cultures grew Mycobacterium tuberculosis in two, yielding the diagnosis in 11 out of the 12 patients. Bacteriological and pathological examinations were non contributive in only one patient. Microbiological examination of pus disclosed two positive direct smear and three positive cultures. Treatment lasted 9 to 18 months. The outcome was favourable in all patients.

[Fracture of the sacrum caused by bone insufficiency in the elderly: apropos of 14 cases]. / Fracture du sacrum par insuffisance osseuse chez le sujet âgé: à propos de 14 observations.

We report a series of 14 elderly patients with sacral insufficiency fractures of the sacrum. All patients presented with pain in low back, pelvis or lower limb which was often misleading. Bone scintigraphy was diagnostic in all the patients. Outcome was favourable in all the cases.

[Pancytopenia in acute brucellosis]. / Pancytopénie au cours d'une brucellose aiguë.

Leuconeutropenia is a common manifestation of acute brucellosis, whereas other hematological abnormalities and pancytopenia are uncommon. We report a patient presenting with acute brucellosis and pancytopenia.