A study of ovarian function in rheumatoid arthritis.

Ovarian function was evaluated prospectively in 16 female rheumatoid arthritis patients in the childbearing age. None of the patients was using oral contraceptives. The control group included 31 women with tubal sterility. Serum levels of estrone, 17 beta-estradiol and progesterone were assayed and used to determine an estrogen index and a luteal index. An ovulatory score was calculated as the sum of the estrogen and luteal indices. Estrogen levels were lower in the rheumatoid arthritis group than in the control group, but the difference was not significant. No statistically significant difference was found for progesterone levels, suggesting that luteal function was normal in the rheumatoid arthritis patients. The rheumatoid arthritis group had a significantly lower mean ovulatory score reflecting a decreased likelihood of ovulation as compared with the control group (p < 0.01). This abnormality may explain why fertility is reduced in women with rheumatoid arthritis.

Focal myositis: a localized form of polymyositis?

We describe an additional case of focal myositis presenting as a painful pseudotumor of the lower leg. Surgical biopsy showed typical features of myositis; the disease improved spontaneously, without clinical or biological abnormalities. Focal myositis can remain a localized process, but in some cases it can become an authentic polymyositis. These generalized forms are associated early with elevated erythrocyte sedimentation rate and/or muscular enzymes.

[Cerebral thrombophlebitis disclosing Behçet disease. Value of high resolution MRI]. / Thrombophlébite cérébrale révélatrice d'une maladie de Behçet. Apport de l'IRM de flux.

A 21-year-old male of Moroccan descent was admitted for inflammatory polyarthralgia and monoarthritis of the left knee. The patient reported a history of recurrent isolated aphthous ulcerations of the oral cavity. He was found to be positive for the HLA B5 antigen. The other investigations for Behçet's disease were negative. During hospitalization, headache responsible for insomnia developed. Neurologic evaluation was normal, as well as funduscopy and spinal tap findings. CT scan images were suggestive of cerebral thrombophlebitis. Magnetic resonance imaging (MRI) disclosed an old thrombosis of the right transverse sinus. Time-of-flight MRI (MRI with sequences adapted to the study of vessels) evidenced recent thrombophlebitis of the left transverse sinus. Cerebral angiography fully confirmed MRI findings. Treatment with a corticosteroid and an anticoagulant was successful. Final diagnosis was Behçet's disease. Subsequently, the patient developed additional features of the disease, including hypersensitivity to pinprick, retinal vasculitis, and erythema nodosum. This case is interesting for two reasons: cerebral thrombophlebitis in Behçet's disease can manifest as localized headache. It occurs in 6 to 13% of patients. Early diagnosis and treatment are essential; MRI features of cerebral thrombophlebitis were confirmed by the cerebral angiogram. MRI established that the thrombophlebitis was of recent onset. MRI may be appropriate as the initial investigation in patients with suspected cerebral thrombophlebitis.

[Rheumatoid aortic insufficiencies: severity of the prognosis]. / Insuffisances aortiques rhumatoïdes: sévérité du pronostic.

Rheumatoid aortic incompetence is infrequent, with only 43 cases published in the literature. Seven additional patients (5 females and 2 males) with rheumatoid arthritis (RA) and aortic incompetence are reported herein. All seven patients had seropositive RA with severe joint disease and extraarticular manifestations (nodules, cutaneous vasculitis, multiple neuritis). Development of aortic incompetence was unrelated to age or duration of RA. The aortic disease was diagnosed upon the development of sudden heart failure (pulmonary edema) in three patients and during a routine evaluation in the other four. The course was extremely severe with a fatal outcome in five patients, of whom the youngest was only 24. Only one patient had valve replacement surgery; however, this patient died 8 days after the procedure. Mean survival in the seven patients was 20 months (range 7-56) from diagnosis and 11 months (range 1-28) from the first manifestation of heart failure. Histologic studies done in the only patient who had surgery demonstrated a rheumatoid granuloma in the pericardium and lymphocytic infiltrates in the pericardium and aortic valve.

[Portal hypertension and hypervitaminosis A. Apropos of 2 cases and review of the literature]. / Hypertension portale et hypervitaminose A. A propos de deux cas et revue de la littérature.

Portal hypertension consecutive to hypervitaminosis A has seldom been well documented. Two cases are reported here. The first case concerns a 39-year old woman who had taken meladinine (8-methoxypsoralen) for the purpose of tanning. In the second case, a 43-year old woman had absorbed Plethoryl (a combination of tiratricol, cyclovalone and retinol) in order to lose weight. Both patients had histological hepatic lesions, such as hypertrophic Ito cells, perisinusoidal cirrhosis and spontaneous autofluorescence, suggestive of vitamin A overload, associated with a hepatic vein pressure gradient of 10 mmHg or more and high liver concentrations of vitamin A. The responsibility of Plethoryl in case 2 is beyond any doubt, but the mechanism of hypervitaminosis in case 1 is controverted. A review of the literature has provided detailed information on the signs of vitamin A overload, the frequency of which is probably underestimated. Patients with portal hypertension of unknown origin should be investigated for hypervitaminosis A.

Immunofixation improves the detection of monoclonal gammopathy of undetermined significance (M.G.U.S.) in patients with rheumatoid arthritis.

Monoclonal gammopathy of undetermined significance (MGUS) was detected by immunofixation (IFX) in 6% of patients with rheumatoid arthritis (RA), whereas it was detected by immunoelectrophoresis in only 1% of these patients. Most of the patients with both RA and MGUS had polyclonal B cell activation, together with their monoclonal band, extra-articular features and long-lasting disease, which suggests a direct relationship between MGUS and RA status.