G Magadur-Joly’s research while affiliated with Centre Hospitalier Universitaire de Nantes and other places

(1) To evaluate the corticosteroid sparing effect of an initial intravenous (i.v.) pulse of methylprednisolone (MP) in the treatment of simple forms of giant cell arteritis (GCA). (2) To analyze corticosteroid response, steroid related side effects, and GCA complications. Patients received a 240 mg i.v. pulse of MP followed by 0.7 mg/kg/day oral prednisone (Group 1) or 0.7 mg/kg/day prednisone without an i.v. pulse (Group 2, controls), or a 240 mg i.v. pulse of MP followed by 0.5 mg/kg/day prednisone (Group 3). Corticosteroid dosage was reduced after normalization of 2 biological inflammatory variables to obtain half-dosage after 4 weeks in Groups 1 and 2 and 20 mg/day after 2 weeks in Group 3. Tapering was systematically attempted from the 6th month of treatment. One hundred sixty-four patients were included in the trial (1992-96). Cumulative doses of corticosteroids after one year were identical for all groups (p = 0.39). No significant differences were observed in the time required for normalization of C-reactive protein, corticosteroid resistance (13.5%), and corticosteroid related side effects (39% of patients; p = 0.37). Corticosteroid resistant patients received larger doses and showed a high risk of GCA related complications (p = 0.02). MP pulses have no significant longterm, corticosteroid sparing effects in the treatment of simple forms of GCA and should be limited to complicated forms. Moreover, corticosteroid resistance is a real risk factor for GCA complications.

The authors report a giant cell arteritis case associating trismus and hemifacial oedema in a febrile context. After spontaneous regression of other manifestations, the apparition of more typical signs allowed to associate the diagnosis of temporal arteritis, later confirmed histologically. Thus, when facing a trismus case, even more when fever is present, it seems important to associate with the Horton's disease, no matter what the antecedents found at the interrogatory be, whether initial or isolated. The Doppler reveals flux abnormalities of the superficial branches of the external carotid. The examination of facial, temporal and internal maxillary arteries has a good negative predictive value in this pathology. It would be useful in therapeutic supervision.

Three men and one woman (mean age 52 years) were admitted to hospital for septicemia (2 cases), sudden partial loss of visual acuity (1 case) and suspected conjunctivitis (1 case). Three of the patients showed risk factors (diabetes, alcohol intoxication, immunosuppression). Panophthalmitis (affecting all tunics of the eye) was apparent from the initial examination in all 4 cases (2 bilateral and 2 unilateral). Ocular involvement was associated with endocarditis and meningitis (pneumococcus) in 1 case, with nocardiosis (pulmonary, cerebral and nodal) in 1 case, and with septicemia with bacterial arthritis (Escherichia coli, streptococcus A) in 2 cases. Hemocultures were positive in 3/4 cases. The micro-organism was also detected in the joint (n = 2), urine (n = 1) and cerebrospinal fluid (n = 1), during pulmonary transparietal puncture (n = 1) and in intraocular biopsy tissue (n = 1). All patients received appropriate antibiotic therapy intravenously and intraocularly. The infection was cured in all cases, but with severe functional sequelae: blindness in 2 cases, and unilateral enucleation in the other 2 cases.

Opinions diverge in the literature as to the predictive value of ANCA measurements rise in relapses of Wegener's granulomatosis. We report a case in which the monitoring of ANCA after remission of Wegener's granulomatosis was misleading. Indeed, positivity in ANCA was related in two instances to a staphylococcal process (osteomyelitis).

Three men and one woman (mean age 52 years) were admitted to hospital for septicemia (2 cases), sudden partial loss of visual acuity (1 case) and suspected conjunctivitis (1 case). Three of the patients showed risk factors (diabetes, alcohol intoxication, immunosuppression). Panophthalmitis (affecting all tunics of the eye) was apparent from the initial examination in all 4 cases (2 bilateral and 2 unilateral). Ocular involvement was associated with endocarditis and meningitis (pneumococcus) in 1 case, with nocardiosis (pulmonary, cerebral and nodal) in 1 case, and with septicemia with bacterial arthritis (Escherichia coli, streptococcus A) in 2 cases. Hemocultures were positive in 3/4 cases. The micro-organism was also detected in the joint (n = 2), urine (n = 1) and cerebrospinal fluid (n = 1), during pulmonary transparietal puncture (n = 1) and in intraocular biopsy tissue (n = 1). All patients received appropriate antibiotic therapy intravenously and intraocularly. The infection was cured in all cases, but with severe functional sequelae: blindness in 2 cases, and unilateral enucleation in the other 2 cases.

To estimate the incidence of adult Still's disease (ASD) and to specify, if possible, associated factors. A retrospective study of the populations of the Brittany and Loire regions in west France was made from 1 January 1982 to 31 December 1991. All internal medicine and rheumatology practitioners of these regions were consulted. Sixty-two (62) cases were reported (93% response). The disease incidence calculated over five years was 0.16 per 100,000 inhabitants in the study population. There was no sex bias (sex ratio 1.06 in ASD v 1.05 in the overall population. The mean age of the study population was 36 years, with two peaks of distribution at 15-25 and 36-45 years. A history of allergy was present in 23% of patients (n = 14). In two patients, it was possible to correlate an environmental allergen to exacerbation of ASD. The yearly incidence of ASD was estimated to be 0.16 per 100,000 inhabitants. However, it was not possible to incriminate any infectious, toxic, or genetic factors in exacerbation of the disease.

The authors report an exceedingly rare complication of rheumatoid arthritis, i.e. acquired hemophilia due to anti-factor VIII autoantibody production. Treatment with intravenous immune globulin ensured control of hemorrhagic manifestations by inducing a transient rise in factor VIII level.

This is a 22-year-old woman's case report. A Wegener disease was discovered in 1989 and treated with success. Eleven months later, ANCA levels routinely doses in serum was increased. It does not reflect a relapse of Wegener disease but a Staphylococus aureus osteomyelitis.