[Non-complicated Horton's disease: initial treatment with methylprednisolone 500 mg/day bolus for three days followed by 20 mg/day prednisone-equivalent. Evaluation of 15 patients]. / Maladie de Horton non compliquée: traitement initial par trois bolus de 500 mg de méthylprednisolone suivis de 20 mg/j d'équivalent-prednisone. Evaluation chez 15 patients.

PURPOSE: To assess the efficacy and tolerance of three methylprednisolone boluses (500 mg/d) followed by a standard dose of prednisolone, 20 mg/d, as the initial treatment of non-complicated giant-cell arteritis. METHOD: A retrospective study of 15 cases. RESULTS: Six men and nine women with a mean age of 70.9 years were treated and followed for 41.5 months. Initial mean ESR was 83 mm; mean C-reactive protein level was 94.6 mg/L. The boluses were well tolerated, excepted in one patient who developed acute psychosis. After initiating the oral treatment, two patients presented signs of clinical relapse during the first month, and were given higher doses of corticosteroids. At 1 month, 12 patients were asymptomatic, nine of whom had normalized ESR and CRP. Mean ESR was 23; mean CRP was 13 mg/L. At 3 months, the mean prednisone dose delivered was 18.2 mg/d. Mean ESR was 12 mm. The cumulative prednisone dose given during the first year was 5,349 (+/- 2,512) mg. In the 13 patients who necessitated no more than 20 mg/d prednisone, no sequelae of giant-cell arteritis, no fractures nor major treatment intolerance occurred-during the first 2 years of treatment. Treatment was stopped in eight patients after a mean duration of 48.6 months. CONCLUSION: Treatment with pulse methylprednisolone 500 mg/d for 3 days followed by 20 mg/d oral prednisone could be a valuable corticosteroid-sparing strategy in many patients with uncomplicated temporal arteritis.

[Treatment of non-complicated Horton's disease]. / Traitement de la maladie de Horton simple.

Non-complicated giant-cell arteritis is defined by the absence of ischemic complications, a remarkable response to corticosteroid treatment, and the absence of corticodependence. It is the most common type of giant-cell arteritis. Treatment relies on corticosteroids (prednisone or prednisolone) at an initial dosage of 0.7 mg/kg/j. The median duration of treatment is 2 years, but many patients need a more prolonged course. Side effects are frequent, especially atherosclerosis and osteoporosis. There is no demonstrated efficiency of corticosteroid sparing agents such as hydroxychloroquine or methotrexate. Dapsone may be efficient, but is not indicated because of its serious side-effects.

Relation between HLA DRB1 alleles and corticosteroid resistance in giant cell arteritis.

OBJECTIVE: To evaluate the clinical usefulness of genomic HLA typing during the first two years of established giant cell arteritis (GCA). METHODS: HLA typing was performed by PCR-SSO in 41 selected white patients with GCA confirmed by biopsy. Patient data were compared with those of a control group of 384 bone marrow donors (relative risk, p value and chi 2 test for each allele). Clinical features at onset and response to treatment over a two year period were evaluated in relation to the genetic pattern. RESULTS: DRB1*04 was significantly increased in the GCA group (frequency of 48.78% compared with 19.79% in controls, p < 0.001). The distribution of the DRB1*04 subtypes in the GCA group was similar to that in controls. No clinical or biological differences were found in association with HLA at the time of diagnosis. Over the two year follow up, nine patients presented resistance to corticosteroid treatment and eight of these (88.88%) had DRB1*04 (p < 0.001). CONCLUSIONS: GCA seems to be associated with HLA DRB1*04 (regardless of the subtype) and this association appears to be accompanied by corticosteroid resistance, suggesting that genomic typing may be useful to identify patients eligible for early alternative treatment to corticosteroid drugs.

Erythrocyte hyperaggregation and thrombogenic dysfibrinogenemia.

Erythrocyte aggregation was measured in 12 patients with congenital dysfibrinogenemia. The results showed hyperaggregation in four patients who had presented a thrombotic disorder, while aggregation was entirely normal in patients with asymptomatic dysfibrinogenemia. None of the four symptomatic patients had any other anomaly of hemostasis, in particular no coagulation inhibitor deficit or anti-phospholipid antibodies. The possible involvement of erythrocyte hyperaggregation in the thrombotic process is discussed.

[Systemic manifestations of primary Gougerot-Sjögren syndrome. Nature and incidence apropos of 34 cases]. / Manifestations systémiques du syndrome de Gougerot-Sjögren primaire. Nature et fréquence à propos de 34 cas.

OBJECTIVES: Assess the systemic manifestations observed in patients with primary Sjörgen's syndrome and the correlation between clinical findings and paraclinical data. METHODS: This retrospective study included patients who met the preliminary European criteria established in 1993. RESULTS: Among the 34 patients with primary Sjögren's syndrome (32 women, 2 men, mean age 54.9 years), systemic manifestations were observed as the inaugural sign in 25 (73.5%) and 31 cases (91%) during a mean 43-month follow-up. Clinical signs in 23 patients were: neuropsychiatric disorders including depressive syndromes (n = 17) and peripheral neuropathies (n = 13), joint and muscle disorders (n = 16), Raynaud's phenomenon (n = 8), pulmonary disorders (n = 8), digestive disorders (n = 8) including two cases of chronic autoimmune hepatitis, skin disease (n = 7), and renal (n = 3) and thyroid (n = 8) disorders. Antinuclear antibodies were found in 20 cases and anti-SSA antibodies in 5 associated with anti-SSB in 4. Eight patients had hematologic disorders. There was a correlation between the number of systemic signs (at least 2) and positive Rose-Bengal test (p = 0.045). DISCUSSION: Different systemic manifestations were found at percentages comparable with data in the literature except for Raynaud's syndrome which was less frequent and neurological disorders which were more frequent.

[Prevalence of hypothyroidism and hyperthyroidism in temporal arteritis and rhizomelic pseudopolyarthritis. A controlled study of 104 cases]. / Prevalence de l'hypothyroidie et de l'hyperthyroidie dans la maladie de Horton et la pseudopolyarthrite rhizomélique. Etude contrôlée portant sur 104 cas.

The aim of this study was to assess the prevalence of hyperthyroidism and hypothyroidism in giant cell arteritis and polymyalgia rheumatica. The prevalence of thyroid dysfunction in giant cell arteritis and polymyalgia rheumatica patients was determined retrospectively from 1976 through 1984 and prospectively from 1984 through 1991. A control group was composed of patients over 55 years of age consecutively admitted to the same hospital department for another condition. Patients were screened for thyroid dysfunction using a thyrotropin assay. Abnormal results were evaluated by T3 and T4 assays and, if needed, a TRH test. Among the 68 giant cell arteritis patients (mean age 72.6 +/- 7 years), of which 41 were included in the prospective arm of the study, 6 had hypothyroidism and 3 had hyperthyroidism. Corresponding figures were 4 and 4 among the 36 patients with polymyalgia rheumatica (mean age 71.7 +/- 8.3 years), of which 18 were evaluated prospectively. Among the 305 controls (mean age 71.6 +/- 9.4 years), 16 had hypothyroidism and 10 had hyperthyroidism. Prevalences of hypothyroidism, hyperthyroidism, and antithyroid antibodies were not significantly different in the control and case groups. Data fail to support previous suggestions that giant cell arteritis or polymyalgia rheumatica patients may be an increased risk for hypothyroidism or hyperthyroidism. They lend no indirect support to the hypothesis that giant cell arteritis and polymyalgia rheumatica may be autoimmune disorders.

[Neoplastic hypercalcemia: prognostic factors of survival of patients; from 51 cases seen in internal medicine]. / Hypercalcémies néoplasiques: facteurs pronostiques pour la survie des patients; à partir de 51 cas observés en médecine interne.

In our Internal Medicine department, we conducted a retrospective study of prognostic factors in patients with malignant hypercalcaemia. The records of 51 patients who had both hypercalcaemia and a histologically proven cancer were analyzed; 42 had a solid tumour and 9 had a myeloma. In 61% of the patients cancer had been revealed by hypercalcaemia. The main warning signs were alteration of the general condition (68.6%), pain in the bones (54.9%) and polyuria with dehydration (58.8%). Osteolysis was observed in 75% of the cases. The overall median survival was 86 days. Patients with myeloma had a significantly longer survival than patients with other tumours (312 versus 60 days; p < 0.05). Patients who had received a causal treatment had a longer survival (176 versus 36 days, p < 0.001). In patients with solid tumours we found a negative correlation between survival and initial calcaemia, and a positive correlation between phosphoraemia, albuminaemia and survival. Multivariate analysis showed that the initial calcaemia level and the possibility of causal treatment were the two cardinal prognostic factors. Although the overall survival rate is mediocre, we believe that hospitalization of patients with malignant hypercalcaemia is justified for their better survival comfort and for the possibility of discovering a neoplasia that could benefit from an effective causal treatment, which is the principal factor of improved prognosis.

[Neurologic and psychiatric manifestations of primary hyperparathyroidism. Study of 5 cases]. / Manifestations neurologiques et psychiatriques de l'hyperparathyroïdie primitive. Etude de 5 cas.

Among 8 of the 20 records of primary hyperparathyroidism examined in search of neurological and/or psychiatric manifestations, the authors extracted 5 clinical cases reported here. These 5 cases were selected because these manifestations had been well evaluated before and after curative surgery of the disease. Among the manifestations reported, there was one corpus striatum syndrome, one cervical myelopathy and three cases of "chronic" psychiatric disorders of several years duration completely cured after parathyroidectomy. The authors underline that such psychiatric disorders should be taken into account in the discussion of treatment.

[Generalized edema caused by licorice: a new syndrome. Apropos of 3 cases]. / Oedèmes généralisés induits par la réglisse: un nouveau syndrome. A propos de 3 observations.

Licorice abuse is a wellknown cause of high blood pressure, myopathy, and cardiac rhythm trouble. It should be considered as a cause of diffuse acute edema, as shown in the three following case-reports.