Efficacy of intravenous gammaglobulin therapy in chronic refractory polymyositis and dermatomyositis: an open study with 20 adult patients.

Purpose: Polymyositis and dermatomyositis are inflammatory muscular diseases of unknown cause. Many interventions are available to treat patients with these conditions including corticosteroids, immunosuppressive drugs, plasmapheresis, and total body irradiation. However, these therapies are not always effective, and they may be associated with certain serious side effects.

[Severe pulmonary artery involvement of Takayasu arteritis. 3 cases and review of the literature].

Three cases of pulmonary arterial disease were identified out of a cohort of 75 cases of Takayasu's disease. In the first case, pulmonary hypertension considered to be idiopathic caused massive haemoptysis and the death of a 48 year old Spanish woman; autopsy revealed characteristic stenoses of the common carotid arteries. In the second case, haemoptysis led to pneumonectomy in a 23 year old West Indian woman with a diagnosis of agenesis of the right pulmonary artery.

[Pulmonary arterial hypertension and systemic diseases].

Certain system diseases which include vascular involvement may be accompanied by pulmonary artery hypertension (PAHT). This chiefly concerns connective tissue disorders, notably scleroderma, CREST syndrome and mixed connective tissue disease and, to a lesser extent, systemic lupus erythematosus. The onset of PAHT in relation to vasculitis of the great vessels or necrosing angeitis is rare.

[Randomized double-blind study of delayed-action verapamil as a single dose versus 2 doses daily in patients with moderate hypertension].

The antihypertensive action of sustained release verapamil is, in the majority of cases, obtained with a dosage of 240 mg per day. In certain patients, it is necessary to increase the daily dose to 360 or even 480 mg. This dosage increase most often leads to twice daily administration.

[Effectiveness of intravenous immunoglobulins in polymyositis and dermatomyositis. An open trial in 15 patients].

Polymyositis (PM) and dermatomyositis (DM) are dysimmune diseases usually treated with corticosteroids and immunosuppressants. Human polyvalent immunoglobulins administered intravenously (IgIV) are known to be effective in some dysimmune diseases. Between August 1987 and September 1989 we conducted an open trial of IgIV in 15 patients (mean age 44 +/- 14 years) with either PM (12 cases) or DM (3 cases) associated with a collagen disease in 2 patients.

[Fenofibrate induced polymyositis].

In a 65-year old man complaining of myalgias and loss of muscular strength, the creatine phosphokinase level was found to be 35 times higher than normal values. The electromyogram was of the diffuse myogenic type. Muscle biopsy showed necrotic lesions of the muscle fibres associated with perivascular infiltrates of mononuclear cells, leading to the diagnosis of polymyositis.

[Vasculitis and neoplasms. 14 cases].

Fourteen cases of vasculitis associated with a neoplasm are reported. The vasculitides were classified as: leukocytolytic vasculitis 7, periarteritis nodosa 4, purpura rheumatica 1, cutaneous granulomatous vasculitis 1, microvasculitis nervosa 1. The neoplasms were diagnosed as: 10 hemopathies (including 4 cases of refractory anemia) and 5 solid tumors (1 patient had 2 tumors).

[Pulmonary hyalinizing granuloma. Apropos of 2 new cases].

Pulmonary hyalinising granuloma are nodular or localised fibrosing lesions of the pulmonary parenchyma and are single or multiple. We report two new cases of this disorder which is rare, as only 62 cases have been published in the literature. It is a pathology with few symptoms, sometimes revealed by general signs.

[Isolated recurrent superficial steroid-induced phlebitis of the lower limbs in young women. A new entity].

The authors report 3 personal cases of recurrent, strictly superficial phlebitis of the lower limbs which developed in young women and responded dramatically to corticosteroid therapy. The phlebitis was oestrogen dependent: it has started during pregnancy and was exacerbated in the second part of the menstrual cycle. Clinically, the disease presented as red, dilated veins and/or isolated inflammatory nodules mimicking acute nodular panniculitis.

[Chronic constrictive pericarditis responsible for an increase of CA 125 levels. Two cases].

Despite its poor specificity, CA 125 is a widely used tumoral marker. Significant rises in serum CA 125 levels are found mainly in ovarian cancer, but they also occur in other malignant or benign pathologies such as chronic liver diseases. However, in benign pathologies the CA 125 concentrations hitherto reported did not exceed 850 U/ml.

[Value of "bolus" cyclophosphamide injections in Behçet's disease. Experience of 17 cases].

Seventeen patients with Behçet's disease were treated with intermittent intravenous bolus injections of cyclophosphamide. Twelve patients had severe eye lesions, 7 had central neurological disorders and 2 had both ocular and central nervous system involvement. Cyclophosphamide was given during 4 weeks on average, in mean doses of 922 +/- 127 mg.

The multiple clinical aspects of lupus.

New insights about the following aspects of lupus erythematosus are developed: (1) thrombotic and neurological manifestations associated with the presence of antiphospholipid antibodies; (2) cardiac complications, especially coronary ischemia, conduction abnormalities and valvular involvement; (3) pregnancy, with particular attention to fetal and maternal prognoses, Soulier-Boffa syndrome and congenital atrioventricular heart blocks; (4) subacute lupus erythematosus, bullous lupus and cutaneous manifestations associated with the lupus anticoagulant.

[Coccygodynia disclosing Tarlov's cysts].

In a 63-year old male patient coccygodynia, initially isolated then complicated by incomplete cauda equina syndrome, could be attributed to large perineurel meningeal cysts on the sacral nerve roots. The diagnosis was suspected at computerized tomography and nuclear magnetic resonance and confirmed by sacculoradiculography. Intradural injections of corticosteroids provided lasting pain relief.