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Prognosis of giant cell arteritis including temporal arteritis and polymyalgia rheumatica. A follow-up study on ninety patients treated with corticosteroids.

Ninety patients with giant cell arteritis (GCA) were followed 3-10 years after the diagnosis. The mean observation time was 63 months. Thirteen patients died. Corticosteroids were administered to all but one patient; 35 were still on treatment after a mean observation period of 59 months. In 38 patients, 94 flare-ups of the disease were recorded during corticosteroid treatment, most of them occurring during the first year of treatment and when a low dose of prednisolone had been given. Thirty-three relapses, 76% within 3 months, occurred in 28 patients after withdrawal of treatment. One patient relapsed after more than ten years of disease. Polymyalgia rheumatica was the most common symptom of flare-up or relapse, regardless of the clinical picture at the time of diagnosis. The duration of treatment should be individualized. One year of treatment is enough in a few patients, whereas others need steroid therapy for more than four years. The rate of intercurrent disease and complications of GCA or its treatment was low. No patient developed severe eye damage due to GCA. The mortality rate was in fact lower than expected with regard to age and sex.

Aged↗

Long-term survival in giant cell arteritis including temporal arteritis and polymyalgia rheumatica. A follow-up study of 90 patients treated with corticosteroids.

Ninety patients with giant cell arteritis (GCA) were followed for 9-16 years after diagnosis. Corticosteroids were given to all but one patient. Forty-two patients died during the observation period (27 women and 15 men). The survival was compared with that in the general population and found not to be reduced among the GCA patients. In fact, we found a lower mortality than expected after five years from diagnosis (p less than 0.05). We did not see any increase in deaths due to vascular or malignant diseases in our patients.

Adrenal Cortex Hormones↗

Visual performance in giant cell arteritis (temporal arteritis) after 1 year of therapy.

AIMS: To determine if patients with giant cell arteritis (GCA) treated with corticosteroids develop delayed visual loss or drug related ocular complications. METHODS: In a multicentre prospective study patients with GCA (using precise diagnostic criteria) had ophthalmic evaluations at predetermined intervals up to 1 year. The dose of corticosteroid was determined by treating physicians, often outside the study, with the daily dose reduced to the equivalent of 30-40 mg of prednisone within 5 weeks. Subsequently, treatment guidelines suggested that the dose be reduced as tolerated or the patient was withdrawn from steroids in a period not less than 6 months. RESULTS: At presentation, of the 22 patients enrolled, seven patients had nine eyes with ischaemic injury. Four eyes had improved visual acuity by two lines or more within 1 month of starting corticosteroids. No patients developed late visual loss as the steroid dose was reduced. At 1 year the visual acuity, contrast sensitivity, colour vision, and threshold perimetry were not significantly different from the 4-5 week determinations. At 1 year, there were no significant cataractous or glaucomatous changes. At 2 months, there was no difference in systemic complications between patients who received conventional dose (60-80 mg per day) or very high doses (200-1000 mg per day) of corticosteroids at the start or early in the course. CONCLUSIONS: Patients with GCA related visual loss can improve with treatment. Corticosteroids with starting doses of 60-1000 mg per day, with reduction to daily doses of 40-50 mg per day given for 4-6 weeks, and gradual dose reduction thereafter, as clinically permitted, did not result in delayed visual loss. There were no significant drug related ophthalmic complications.

Adrenal Cortex Hormones↗

[Necrosis and ischemia of the femoral head during arteritis of the lower extremities. Survey of 138 patients with arteritis and 159 aortographies].

Among 138 subjects suffering form arteritis of the lower limbs, the authors found clinical signs of lesions in one or both hips in 47.6 percent of cases and radiological signs in one or both hips in 42.8 percent of cases. Five patients presented a distinct flattening of the femoral head indicating necrosis at stage III, 19 showed voluminous or multiple dense islets in the femoral collar or head indicating necrotic lesions at stage II, and 26 showed narrowing of the superior joint space mostly on the internal side. The authors think ihat the latter sign indicates ischaemic chondropathy. Eleven of the 138 patients had undergone pertrochanteric phlebography : this was abnormal in all 11 cases. At the same time the authors studied the coxofemorals on 159 aortograms and found, among other anomalies, 2 cases of advanced necrosis at the stage of rupture (stage III). This study tends to confirm the occurrence of necrotic lesions of the femur that are secondary to arteriopathies.

Adult↗

[Arteritis of the large vessels originating at the aorta (Takayasu arteritis) in childhood (author's transl)].

A 9-month-old female child with extensive inflammatory lesions in the large arterial trunks originating at the aorta (Takayasu arteritis) was reported. Severe cerebral, intestinal and coronary circulatory disturbances as well as insufficient blood supply in both arms, particularly the left arm, developed as a result of the stenoses and obliterations. Clinical course, postmortem findings and the results of EKG, EEG and carotid angiography were discussed. The onset in infancy and the very rapid progression of the disease were unusual.

Aortic Arch Syndromes↗

Aortic arch arteritis in the elderly. An important manifestation of giant cell arteritis.

Five elderly patients with aortic arch syndrome secondary to large-vessel vasculitis all presented with upper-extremity claudication and absence of blood pressure and pulses in the affected extremity. Diagnosis was suspected by a markedly elevated erythrocyte sedimentation rate associated with constitutional symptoms and angiograms showing changes consistent with arteritis of the aortic arch vessels. Treatment with high-dose corticosteroids resulted in rapid resolution of constitutional symptoms and improved functional use of the upper extremity. Recognition of this steroid-responsive disease is essential to avoid unnecessary reconstructive surgery and to prevent catastrophic events such as aortic dissection or visual loss, which may result from the underlying disease process.

Adrenal Cortex Hormones↗