Prognostic factors and long-term evolution in a cohort of 133 patients with giant cell arteritis.

OBJECTIVE: Survival in patients with giant cell arteritis (GCA) has generally been found to be similar to that of the general population. The aim of our study was to assess outcome and survival of different subgroups of patients with GCA in relation to clinical, biological data or treatment modalities. METHODS: From 1977 and 1995, 176 patients were treated in the Department of Internal Medicine for GCA. The patient, family or local practitioner were contacted prior to the study (July-October 1995). Treatment modalities and follow-up were obtained for 133 patients. All patients (except 11) had 3 or more 1990 ACR classification criteria for GCA. The 11 patients with 2 criteria had a positive temporal biopsy and were included in the study. RESULTS: Relapse during corticosteroid tapering treatment was observed in 83 patients (62.4%) with a mean 1.57 relapses per patient. No correlation was found in age, sex, initial dose or type of steroid used (i.e. prednisone or prednisolone). Only a slight correlation in the initial erythrocyte sedimentation rate (ESR) was observed (p < 0.01, r = 0.23). In 56 patients free of treatment (mean treatment duration: 40 months), 27 (48%) developed a relapse of the disease 1 to 25 months later. No correlation was found in age, sex, initial dose of steroid, number of relapses during treatment, or initial ESR. Survival analysis was performed using the Kaplan-Meier and Mantel-Menszel methods for comparison of groups. At the time of the study, 41 patients had died (30.7%). A significant reduction of survival was found with the presence of permanent visual loss vs absence (p = 0.04), in patients who required more than 10 mg/d of glucocorticoid (p < 0.001) at 6 months treatment and in patients treated with prednisone (vs prednisolone) (p < 0.01). However, these factors were not independently associated with survival in the multivariate analysis. CONCLUSION: Relapse was observed in 62.4% of the patients during corticosteroid tapering (correlated with initial ESR). A relapse of the disease was also observed in 48% of patients 1 to 25 months after the end of the treatment and was associated with prednisolone use. Long term survival was better in patients with no initial ocular manifestations, in patients who took less than 10 mg/day of corticosteroids at 6 months of the treatment and in patients treated with prednisolone.

[What vascular events suggest a myeloproliferative disorder?]. / Devant quelles manifestations vasculaires rechercher un syndrome myéloprolifératif?

Patients with polycethemia vera (PV) or essential thrombocythemia (ET) are at increased risk of arterial and venous thromboembolic events. Arterial ischemic complications occur in 24 to 43% of these patients, particularly those with cardiovascular risk factors (especially cigarette smoking). Non-atheromatous arterial thrombosis concerns all large and medium-sized vessels, particularly cerebral, limb, coronary and digestive arteries. Extensive complications have been described in patients with lower limb occlusive arteriopathy, particularly stent or bypass thrombosis, critical ischemia. Juvenile myocardial infarction or rapid postangioplasty coronary thrombosis may reveal certain myeloproliferative disorders, particularly ET. Venous thrombosis is more frequent in PV than in ET; superficial or deep venous thromboses are seen. Thromboembolic events occur in about 25-30% of the patients and account for one-third of the deaths. Mesenteric vein thrombosis, portal thrombosis, or suprahepatic vein thrombosis may occur in all myeloproliferative disorders, but the pathogenesis is not fully understood. Pulmonary hypertension may be the consequence of local thrombosis in the pulmonary vasculature or may be due to the high blood flow in the right heart cavities. Microvascular circulatory disturbance includes erythromelalgia, Raynaud's phenomenon, digital ischemia, acrocyanosis, blue toe syndrome, livedo reticularis, cutaneous ulcers or necrotic purpura. All these manifestations may precede the myeloproliferative disorder by several months.

[Pyostomatitis-pyodermatitis vegetans uncovering a case of Crohn disease]. / Pyostomatite-pyodermite végétante révélatrice d'une maladie de Crohn.

BACKGROUND: Pyostomatitis-pyodermatitis vegetans is an uncommon condition associated with chronic inflammatory bowel disease in 75% of the cases, usually hemorrhagic rectocolitis. CASE REPORT: A 48-year-old man was referred for recent development of pustulous lesions of the lips and buccal mucosa and weight loss. He complained of abdominal pain and intermittent diarrhea which had persisted for more than one year. During the last three months, a pseudotumoral plaque with a pustulous rim had developed over the two distal phalanxes of the right middle finger in association with ungueal lysis and nodular, vegetating and crusted lesions on the lateral aspect of the left arm. Small pustules covered the entire buccal mucosa excepting the tongue and the glans forming a typical snail trace aspect. Bacterial and mycological samples were negative. The histology reports for skin and mucosa were similar: epithelial hyperplasia, intra- and subepithelial granulocyte micro-abscesses and polymorphous infiltration of the superficial derma with numerous neutrophils and eosinophils. There was a discrete interkeratinocytic fluorescence at direct immunofluorescence but indirect immunofluorescence was negative. Anti-desmogleine 1 immunolabeling showed typical normal skin uptake and immunotransfer was negative. Digestive tract endoscopy and histopathology examination of the bowel specimens confirmed the diagnosis of Crohn's disease. Clinical manifestations improved dramatically with prednisone. DISCUSSION: This case of pyostomatitis-pyodermatitis vegetans involved several aspects rarely reported in the literature: a) the cutaneomucosal signs were inaugural; b) the association with Crohn's disease; c) the presence of lesions to the genital mucosa; d) the unusual localization of the inaugural skin manifestations. This clinical entity has now been clearly distinguished from pemphigus vegetans. There was however a long debate on the similar clinical, histological and even immunological expressions. We suggest that pyostomatitis-pyodermatitis vegetans belongs to the spectrum of neutrophilic dermatoses and other authors even propose it is a clinical form of pyoderma gangrenosum.