Idiopathic hypertrophic pachymeningitis.

BACKGROUND: Hypertrophic pachymeningitis is an uncommon disorder that causes a localized or diffuse thickening of the dura mater and has been associated with rheumatoid arthritis, syphilis, Wegener's granulomatosis, tuberculosis, and cancer. Few series of the idiopathic variety have been described, particularly with respect to MRI correlation to clinical outcome and treatment. OBJECTIVE: To investigate the clinical and laboratory evaluation, course, and treatment of patients with idiopathic hypertrophic pachymeningitis (IHP), to correlate the MRI findings with the clinical course, and to review the literature on IHP. METHODS: Retrospective case series of 12 patients (9 men, 3 women), with a mean age of 55 years (range 39 to 88 years), who had IHP by imaging studies, meningeal or orbital biopsy, or both. The clinical features, laboratory evaluation, contrast-enhanced MRI, treatment, and clinical outcome were documented for each case. The mean duration of follow-up was 3.5 years (range 3 months to 16 years). RESULTS: The main clinical features at presentation were headache (11 cases), loss of vision (7 cases), diplopia (4 cases), papilledema (2 cases), other cranial nerve involvement (3 cases), ataxia (2 cases), and seizures (1 case). On the initial MRI, the location of abnormal enhancement of the dura mater correlated with the clinical findings and the sphenoid wing area was affected in all patients. The sedimentation rate was elevated in five cases. The CSF had increased protein in six cases and lymphocytosis in four cases. Biopsy of the dura mater in five cases and the orbital soft tissue in one case showed infiltrates of small mature lymphocytes, plasma cells, and epithelioid histiocytes, but no neoplasia, vasculitis, or infectious agents. Cultures of the CSF and biopsy material remained sterile. Corticosteroid therapy improved the vision in 7 of 8 cases and controlled headache in 10 of 11 cases. Five cases had partial improvement of other neurologic symptoms and signs. Recurrence developed with steroid tapering in six cases. One case had progressive deterioration and died. In four cases methotrexate or azathioprine was added with reduction of the steroid dose. Follow-up MRI performed in 11 patients correlated 80% with the clinical state (p = 0.01). CONCLUSION: IHP can be suspected on MRI and defined pathologically on biopsy. Untreated, the clinical course is usually marked by severe headache and progressive neurologic deterioration and vision loss. Although initially steroid-responsive, clinical manifestations frequently recur with corticosteroid taper, requiring the addition of immunosuppressive agents in some cases.

A prospective, double-blind, randomized, placebo controlled trial of methotrexate in the treatment of giant cell arteritis (GCA).

OBJECTIVE: To determine if methotrexate has disease-controlling and corticosteroid (cs)-sparing effects in the treatment of giant cell arteritis (GCA). METHODS: This was a randomized, controlled, double-blind trial comparing methotrexate versus placebo in addition to corticosteroid therapy in patients with newly diagnosed giant cell arteritis. Patients with giant cell arteritis were enrolled and treated with high dose corticosteroids as well as methotrexate starting at 7.5 mg/week or placebo. Corticosteroids were tapered by the treating physician as guided by the clinical picture, with methotrexate or placebo dose increased by 2.5 mg/week for disease flare with a maximum allowable dose of 20 mg/week. After a clinically-defined remission and steroid discontinuation, methotrexate or placebo was tapered monthly to zero by 2.5 mg/week. RESULTS: Twenty-one patients were enrolled, 12 randomized to methotrexate, 9 to placebo. Baseline characteristics (age, height, weight, sedimentation rate, bone mineral density, total corticosteroid dose prior to randomization, and quality of life as measured by SF-36 and function as measured by AIMS) were comparable between groups. At completion, there was no significant difference between methotrexate- and placebo-treated patients with regard to the cumulative corticosteroid dose (6469 mg and 5908 mg respectively, p = 0.6), number of weeks to completion of steroids (68 and 60 respectively, p = 0.5), time (weeks) to taper prednisone to less than 10 mg prednisone/day (23 and 25 respectively, p = 0.5), bone mineral density in lumbar spine (p = 0.2) or hip (p = 0.4) at one year, or functional status as measured by AIMS and quality of life as measured by SF36. There was no late vision loss in either group, and only one major treatment-responsive relapse in a methotrexate-treated patient. There were few major corticosteroid-related side effects and these did not significantly differ between groups. CONCLUSION: With this study design, no corticosteroid-sparing benefit could be attributed to the combination of methotrexate with corticosteroid therapy for the treatment of patients with giant cell arteritis. Both groups did well, with few major corticosteroid-related side effects, and most patients were safely tapered off corticosteroids sooner than reported in many series. The shorter overall duration of steroid treatment in this study probably contributed to the remarkably low frequency of side effects, without increased ischemic risk for the patient.

Paraneoplastic rheumatic syndromes.

Paraneoplastic rheumatic syndromes are challenging from both a clinical and research standpoint. Progress over the past decade has provided clarification of clinical syndromes. At the same time, our increasing ability to define and quantitate mediators of inflammation is shedding new light on pathogenesis. In turn, this understanding may answer questions regarding more common rheumatic diseases.

Behçet-type vasculopathy in a patient without the diagnostic features of Behçet's disease.

Behçet's disease is a multisystem inflammatory disorder which may involve the vascular system. The vasculopathy of Behçet's disease is distinctive among the vasculitides in that it involves both arteries and veins, and vessels of all sizes. Most published diagnostic criteria for Behçet's disease include the classic triad of orogenital ulceration and ocular inflammation. In this report, we describe a patient who had a vasculopathy fitting the Behçet's disease type, but who lacked the other characteristic or diagnostic features of Behçet's disease. This case illustrates an unusual presentation and natural history of a complex vasculitic disease.

Recovery from pulmonary hypertension in an adolescent with mixed connective tissue disease.

This paper describes the case of an 11 year old girl who presented with mixed connective tissue disease which was complicated by the development of pulmonary hypertension. This case is unique with respect to the young age of onset, the serial non-invasive method used to follow the disease process, and the favourable response to treatment with vasodilator and anti-inflammatory drugs.

Antemortem diagnosis of giant cell aortitis.

A 67-year-old man well while receiving steroid therapy for giant cell arteritis, developed symptoms of ischemic heart disease and was found to have critical left main coronary stenosis. An aortic biopsy at the time of bypass grafting revealed giant cell aortitis. We discuss the finding of clinically discordant aortitis; the etiology of the concurrent coronary stenosis and therapy with prednisone-dapsone.

Proliferative changes of the hip in juvenile rheumatoid arthritis.

A ring of proliferative osteophytes at the junction of the femoral head and neck has been described as a characteristic finding in patients with ankylosing spondylitis. A review of 55 cases of juvenile rheumatoid arthritis, without evidence of sacroiliitis, revealed an identical ridge of osteophytes in 5.