Disseminated aseptic abscesses associated with Crohn's disease: a new entity?

Our purpose is to describe seven cases of disseminated aseptic abscesses with regard to clinical, biological, radiological, and histological information, treatment, and outcome. Data were collected on seven Caucasian patients who had proven sterile deep abscesses diagnosed in French university hospitals. The onset of the disease related to abscesses began at times from June 1988 to August 1994. Follow-up periods were 1 year, 7 months to 8 years, 2 months. The age of the patients ranged from 15 to 26 years old. At onset, all had fever and six had abdominal pain. Abscesses involved spleen and abdominal lymph nodes in six cases; liver in three; pancreas, brain, and chest in one. All had polymorphonuclear leukocytosis. Pathological examination showed granulomatous abscesses. Direct and indirect investigations failed to identify any causal microorganism. On six occasions, Crohn's disease was revealed 1 to 41 months later and in one case, it preceded the onset of abscesses. One subsequently developed Sweet's syndrome. Various antibiotic regimes were inefficient. Steroids, associated in three cases with immunosuppressive agents, resulted in a rapid improvement in six patients. In one case, splenectomy followed by 5-ASA therapy was used successfully. The dramatic effectiveness of steroids and immunosuppressive agents as well as follow-up suggest that disseminated aseptic abscesses might be an extraintestinal manifestation of Crohn's disease. Although the pathogenesis of this condition remains unknown, this entity may be related to neutrophilic dermatosis in which sterile deep abscesses have been reported.

[Mechanisms of cardiac involvement in systemic scleroderma. Apropos of 3 cases]. / Mécanismes de l'atteinte cardiaque dans la sclérodermie systémique. A propos de trois observations.

To investigate myocardial manifestations in progressive systemic sclerosis, we studied three patients with transthoracic echocardiography, cardiac catheterization with coronary angiography, thallium scans with dipyridamol completed by thoracic cold exposure and endomyocardial biopsy with immunohistology. Two patients were symptomatic. In the three cases, abnormalities of myocardial perfusion were detected without coronary stenosis. Two patients had myocardial fibrosis with a coronary spasm in one case. The immunohistological study always showed an unusual expression of HLA class II antigen on cardiac fibroblasts.

Serum erythropoietin and reticulocyte counts in inflammatory process.

The role of erythropoietin in the pathogenesis of anaemia associated with inflammatory disorders is unclear. We studied serum erythropoietin levels in patients with inflammatory process of varying aetiologies. Serum erythropoietin levels and reticulocyte counts were prospectively measured in 40 patients with inflammatory syndromes and compared with values obtained in 20 patients with myelodysplastic syndromes. Significant inverse correlation between erythropoietin levels and haemoglobin concentration were noted in the 2 groups. The slope of the regression line for patients with inflammatory disorders was lower as compared with that for the myelodysplastic syndromes. In all cases, when the erythropoietin response in relation to degree of anaemia is compared with that which occurs in patients with myelodysplastic syndromes, the inadequate erythropoietin response in patients with chronic inflammatory process becomes evident. In patients with inflammatory process, a relationship between erythropoietin levels and reticulocyte counts were only noted in patients with mildly anaemia (haemoglobin concentration higher than 10.5 g/dl). This study suggests blunted erythropoietin production and impaired marrow response to this hormone in the anaemia which occurs in inflammatory syndromes and supports the hypothesis that these disorders may contribute to the development of the anaemia associated with inflammatory disorders.

[Semi-invasive diffuse pulmonary aspergillosis with antineutrophil cytoplasmic antibodies. 2 cases]. / Aspergillose pulmonaire semi-invasive avec anticorps anti-cytoplasme des polynucléaires de type diffus. Deux observations.

We report two cases of semi-invasive pulmonary aspergillosis with a thoracic wall extension in the first case and a parietal vasculitis without Aspergillus invasion in the second. Semi-invasive pulmonary aspergillosis is an identifiable entity. It is characterized by impairment of local pulmonary defense mechanisms and/or a mild systemic immunosuppression as well as a slowly progressive course. Pulmonary symptoms are not specific. The thoracic wall involvement subsequent to a contiguous pulmonary lesion occurs rarely and indicates poor prognosis. CT scan improves diagnosis significantly, confirmed by histology and/or by the biopsy growth of Aspergillus. Itraconazole, tolerated better than amphotericin B, is an efficient treatment though prognosis remains poor (28% of deaths). Detection of anti-neutrophil cytoplasmic antibodies (diffuse cytoplasmic staining) evolving conversely to this disease seems to be associated with infectious vasculitis. These antibodies are found in vasculitis and especially in Wegener's granulomatosis with a high specificity. They have also been described in a few patients with infectious diseases.

[Painful chronic thyroiditis. A rare cause of thyroid pain]. / Thyroïdite chronique douloureuse. Une cause rare de douleur thyroïdienne.

The usual clinical presentation of Hashimoto's chronic lymphocytic thyroiditis is a firm and painless goiter. We report two atypical observations with painful symptoms. Case 1: a 33 year old woman with a two months history of L-thyroxine treated primary hypothyroidism associated with elevated antithyroglobulin and antithyroperoxydase antibodies (respectively 36.000 and 7.000 U/l, normal values < 100 U/l) presents with anterior cervical pain. She is euthyroid, but displays biological inflammatory syndrome. Neck ultrasonography reveals low amplitude echoes of the thyroid gland. Antithyroid antibodies remain at high titers. After failure of NSAI drugs, prednisolone (oral 30 mg/day for 10 days, followed by degression) allows rapid disappearance of both pain inflammatory syndrome. When treatment was stopped pain recurred and required steroids for 3 months. After one year, the patient is painless and only receives L-thyroxine. Case 2: a 36 year old woman is referred for anterior neck pain, fever and hypothyroidism. Biological inflammation is also noted. High levels of antithyroglobulin and antithyroperoxydase antibodies are found. Thyroid uptake was maintained. L-thyroxine and prednisolone (oral 20 mg/day) therapies are associated with rapid pain relief. After two months, steroids are stopped. The patient remains normothyroid under L-thyroxine. Antithyroid antibodies persist at high levels. Other aetiological diagnosis of painful thyroid include acute infectious thyroiditis, cancers, intracystic hemorrhages and mainly subacute De Quervain's thyroiditis. Beyond clinical symptoms (revelation with hypothyroidism), persistent scintiscan uptake and prolonged elevated levels of antithyroglobulin and antithyroperoxydase antibodies remain the strongest arguments in favor of the 33 reported cases of painful Hashimoto's chronic lymphocytic thyroiditis. Eventually, among various therapies used, steroids in conjunction with thyroid hormones, seem the most efficient.

[Mechanisms of cardiac involvement in systemic scleroderma, apropos of 3 cases]. / Mécanismes de l'atteinte cardiaque dans la sclérodermie systémique (à propos de trois observations).

We studied myocardial manifestations of systemic sclerosis in three patients. Two patients were symptomatic. A transthoracic echocardiography, a coronary angiography, a cardiac catheterization, thallium scans with dipyridamol completed by thoracic cold exposure and endomyocardial biopsy with immunohistological study were performed. None of them have coronary stenosis. In the three cases abnormalities of myocardial perfusion were detected, two of them have fibrosis. The immunohistological study always showed an anusual expression of HLA DR on cardiac fibroblasts.

[Antiprothrombinase and myocardial infarction. Apropos of 2 cases]. / Antiprothrombinase et infarctus du myocarde. A propos de deux observations.

Among the thrombotic events associated with a circulating anticoagulant of the antiprothrombinase type, myocardial infarction is exceptionally reported, which justifies the presentation of two cases. In both patients, myocardial necrosis occurred some time after the antiprothrombinase was discovered, and there was nothing special in its clinical features. No obvious atherosclerotic lesion and no image suggestive of vasculitis were found at coronary arteriography, which suggested that the antiprothrombinase played a predominant role in the genesis of infarction. Relationships between antiprothrombinase, arterial thrombosis (particularly of the coronary arteries) and the presence or absence of systemic lupus erythematosus (SLE) are discussed. As observed in thrombosis of other arteries, it is not certain that the presence of SLE constitutes an additional risk factor, except in cases with unquestionable vasculitis or if the treatment of SLE requires prolonged corticosteroid therapy. On the other hand, the appearance of an antibody directed against phospholipids is not necessarily related to the presence of SLE; in fact, this antibody itself might be a risk factor of myocardial infarction, as has recently been suggested.

[Association of streptococcal spondylitis and endocarditis. 2 new cases]. / Association de spondylodiscite à streptocoque et d'endocardite. Deux nouvelles observations.

Two new cases of spondylitis associated with endocarditis are reported. Approximately 30 cases have already been published in the medical literature. Although this association is infrequent, the possibility of its occurrence calls for repeated auscultation in patients with spondylitis and focal roentgenograms in patient with bacterial endocarditis who develop vertebral pain.