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Biomedical subjects

S Herson

Publications and source records attributed to S Herson.

At least 19 recordsLinked to original sources
PubMed2003

[Myopericarditis during Horton disease].

INTRODUCTION: Myopericarditis is an rare form of Horton disease. We report two new cases. METHOD: Two patients, more than 60-years-old, had developed global cardiac insufficiency with predominant left insufficiency, with physical alteration and biological inflammation. Echocardiography revealed a myopericarditis. Bacteriological explorations were all negative. In the two cases, temporal artery biopsy revealed giant cell arteritis. Treatment with oral corticosteroids improved biological and clinical symptoms. CONCLUSION: Myopericarditis is a rare form of Horton disease. Classical clinical presentation is rarely associated. Temporal artery biopsy is essential in confirming and proposing a specific therapy, in order to prevent cardiovascular ischemic complications.

Adrenal Cortex Hormones↗
PubMed2002

Interstitial lung disease in polymyositis and dermatomyositis.

OBJECTIVES: To assess prevalence, characteristics, and long-term outcome of interstitial lung disease (ILD) in polymyositis (PM) and dermatomyositis (DM). To determine predictive variables of ILD course in PM/DM, and to define both clinical and biochemical features associated with ILD onset in PM/DM. METHODS: The medical records of 156 consecutive PM/DM patients in 3 medical centers were reviewed. RESULTS: Thirty-six PM/DM patients (23.1%) developed ILD. We observed that 19.4% of patients with ILD had resolution of pulmonary disorders, whereas 25% experienced ILD deterioration. Morbidity and mortality rates were as high as 13.9% and 36.4%, respectively, in PM/DM patients with ILD. Parameters of PM/DM that related to ILD poor outcome were identified as follows: Hamman-Rich-like pattern, initial diffusing capacity of carbon monoxide <45%, neutrophil alveolitis, and histologic usual interstitial pneumonia. Additionally, for the group with ILD, polyarthritis, higher values of erythrocyte sedimentation rate and C-reactive protein, presence of anti-Jo-1 antibody, and characteristic microangiopathy were significantly more frequent. CONCLUSION: Our series underlines the high frequency of ILD in PM/DM patients, resulting in increased morbidity and mortality rates. It also indicates that PM/DM patients should routinely be screened for ILD, even those patients without anti-Jo-1 antibody, because 69% of our ILD patients were seronegative for the anti-Jo-1 antibody. Our findings further suggest that PM/DM patients presenting with factors predictive of ILD poor outcome may require more aggressive therapy.

Antibodies, Antinuclear↗
PubMed2002

Results and long-term followup of intravenous immunoglobulin infusions in chronic, refractory polymyositis: an open study with thirty-five adult patients.

OBJECTIVE: Polymyositis is a rare inflammatory muscular disease of unknown cause. Corticosteroids and immunosuppressive drugs are the first choice of therapy but are not always effective and may cause serious side effects. Many studies have shown that polyvalent intravenous immunoglobulin (IVIG) may be of interest for the treatment of dermatomyositis. We carried out an open, prospective study to evaluate the efficacy of IVIG in subjects with polymyositis that was refractory to traditional treatments, and we evaluated the benefits of this therapy over a long-term period of followup. METHODS: Thirty-five adult white patients (20 female, 15 male, mean age 43.5 years [SD 16.8]) with chronic, refractory polymyositis were treated with high doses of IVIG, after the patients had received the following traditional treatments: prednisone (n = 35), methotrexate (n = 24), azathioprine (n = 13), cyclophosphamide (n = 4), cyclosporine (n = 7), chlorambucil (n = 1), plasmapheresis (n = 8), lymphopheresis (n = 1), and total body irradiation (n = 1). There had been no changes in the patients' treatment in the 2 months before the initiation of IVIG therapy, and doses were not increased during IVIG treatment. We used preparations of polyvalent human IVIG with increased concentrations of intact IgG. The patients received 1 gm/kg/day for 2 consecutive days per month. The mean course of treatment was 4-6 months. The clinical assessment involved the evaluation of proximal muscle power, muscle disability scale score, and esophageal disorders. The biochemical evaluations carried out before each treatment period were compared by Student's t-test and nonparametric Wilcoxon test. Results were considered to be significant at P = 0.05. RESULTS: In the short-term, significant clinical improvement was noted in 25 of the 35 patients (71.4%). Mean muscle power was estimated before and after IVIG therapy and was found to be significantly improved (P < 0.01). All patients had a significant biochemical response. Mean creatine kinase levels during IVIG therapy decreased significantly before the fourth IVIG perfusion (P < 0.01). Side effects, usually minor, were noted in 6 patients. This benefit allowed the initial prednisone dose to be reduced by >50% in all patients. The mean (+/- SD) followup time for the 25 patients who responded favorably to IVIG treatment was 51.4 +/- 13.1 months. Twelve of these 25 patients remained in full remission following their initial course of IVIG, resulting in complete stoppage of medication in 5 patients or low doses of steroids in 7 patients. The condition of 6 patients remained improved and no other drugs were prescribed, but the patients remained dependent on IVIG infusions. Seven of the 25 patients who responded well to IVIG treatment relapsed at an average of 17.1 months (range 4-23 months) after the discontinuation of IVIG. CONCLUSION: IVIG is an interesting therapy for the treatment of polymyositis, with results showing that the condition of approximately 70% of the patients tested improved. After the discontinuation of the IVIG therapy, the efficacy remained stable in 50% of the patients, with a followup of over 3 years.

Adult↗
PubMed2001

Severe perturbations of the blood T cell repertoire in polymyositis, but not dermatomyositis patients.

Polymyositis and dermatomyositis are diseases characterized by muscle weakness and muscle inflammatory infiltrates. Their pathogenesis remains unclear. A central role for endomysial autoaggressive CD8(+) T cells is suspected in polymyositis and for perivascular B cells in dermatomyositis. We compared the T cell repertoire of 10 polymyositis and 10 dermatomyositis patients by immunoscope, a method providing a global assessment of the T cell repertoire and a sensitive detection of clonal T cell expansions. Samples were analyzed qualitatively and quantitatively in the blood (unsorted cells and CD4(+) and CD8(+) cells) and in muscle infiltrates. Dramatic perturbations of the T cell repertoire were observed in the blood of polymyositis but not dermatomyositis patients (p < 0.0005), the latter being undistinguishable from controls. These perturbations were due to oligoclonal expansions of CD8(+) T cells and most blood clonal expansions were also found in muscle. These results indicate that the pathogenesis of polymyositis and dermatomyositis is different and reinforce the view that polymyositis but not dermatomyositis is an autoimmune CD8(+) T cell-mediated disease. Moreover, this method may be helpful for the differential diagnosis of polymyositis and dermatomyositis and for noninvasive follow-up of polymyositis patients.

Adult↗
PubMed2001

Successful percutaneous dihydrotestosterone treatment of gynecomastia occurring during highly active antiretroviral therapy: four cases and a review of the literature.

Fourteen cases of gynecomastia occurring during highly active antiretroviral therapy (HAART) have been reported in the literature. To date, no specific therapeutic approach has been proposed, and gynecomastia has usually persisted. We report 4 new cases of HAART-induced gynecomastia that were successfully treated with percutaneous dihydrotestosterone gel.

Administration, Topical↗
PubMed2001

Clinical and spectroscopic improvement in HIV-associated cognitive impairment.

To assess the impact of highly active antiretroviral therapy (HAART) on AIDS-associated cognitive impairment, 22 patients with AIDS with (n = 11) and without (n = 11) cognitive deficit were evaluated clinically and by MRS every 3 months for 9 months. Nineteen patients were on HAART at study entry, 21 after 2 months. Cognitively impaired patients presented with a subcorticofrontal deficit and decreased N-acetyl-aspartate in frontal white matter. These clinical and metabolic abnormalities reversed partially on HAART, whereas they remained within normal limits in cognitively unimpaired patients.

AIDS Dementia Complex↗
PubMed2001

Macrophagic myofasciitis associated with inclusion body myositis: a report of three cases.

We describe three patients with macrophagic myofasciitis and inclusion body myositis. All patients fulfilled diagnostic criteria for inclusion body myositis and myopathologic criteria for macrophagic myofasciitis. In the three cases macrophagic myofasciitis complicated the evolution of a known and painless inclusion body myositis and was diagnosed in a repeated deltoid biopsy because of the appearance of myalgia during the course of inclusion body myositis in all cases. The unexpected appearance of myalgia during the course of painless inclusion body myositis must arouse the suspicion of an association of another inflammatory muscle disease, macrophagic myofasciitis.

Adult↗
PubMed2001

[Methylprednisolone-induced acute generalized exanthematous pustulosis].

BACKGROUND: Acute generalized exanthematous pustulosis is a rare drug allergy. Generalized reactions to systematically administrated corticosteroids are even rarer. We report the first case of acute generalized exanthematous pustulosis due to methylprednisolone. CASE REPORT: A thirty year-old-woman presented, a few hours after intravenous administration of methylprednisolone indicated for multiple sclerosis, a maculopapulous rash predominant in the folds rapidly becoming pustulous with malaise, fever and neutrophilia. The histologic examination and negativity of microbiological cultures were consistent with the diagnostic of acute generalized exanthematous pustulosis. The rash cleared spontaneously in one week with normalization of the biology. One month later, epicutaneous tests, confirmed the allergy to group A corticosteroids. The treatment of multiple sclerosis was pursued with dexamethasone. DISCUSSION: Clinical and histological manifestations were consistent with the diagnostic of acute generalized exanthematous pustulosis to methylprednisolone. Generalized reaction to systematically administered corticosteroids are very rare. Immediate reactions are the most frequently reported reactions, only about thirty delayed-type generalized skin eruptions have been reported to date. Group A corticosteroids are the most frequent causal agent. Epicutaneous tests have good sensitivity for acute generalized exanthematous pustulosis, for allergy to corticosteroids, delayed results are very important.

Acute Disease↗
PubMed2000

[Macrophagic myofasciitis. Study and Research Group on Acquired and Dysimmunity-related muscular diseases (GERMMAD)].

UNLABELLED: MACROPHAGIC MYOFASCIITIS: A most unusual inflammatory myopathy, first described by Germmad had been reported with increasing frequency since 1993 in the leading French myopathology centers. We present our experience with this new disease: macrophagic myofasciitis. CLINICAL FEATURES: By November 1999, 70 cases of macrophagic myofasciitis had been recorded since our first description. The first 22 patients (sex ratio M/F = 1:3) referred with the presumptive diagnosis of polymyositis (n = 11), polymyalgia rheumatica (n = 5), mitochondrial cytopathy (n = 4), and congenital myopathy or muscle dystrophy (n = 1 each). Symptoms included myalgia (91%), anthralgia (68%), marked asthenia (55%), muscle weakness (45%), and fever (32%). LABORATORY FINDINGS: Abnormal laboratory findings included elevated CK levels (50%), markedly increased erythrocyte sedimentation rate (37%), and myopathic EMG (35%). Muscle biopsy showed a unique myopathological pattern characterized by: i) centripetal infiltration of epimysium, perimysium and perifascicular endomysium by sheets of large cells of the monocyte/macrophage lineage (CD68+, CD1a-, S100-, with a PAS-positive content; ii) absence of necrosis, of both epithelioid and giant cells, and of mitotic figures; iii) presence of occasional CD8+ T-cells; iv) inconspicuous muscle fiber damage. The picture was easily distinguishable from sarcoid myopathy and fasciitis-panniculitis syndromes. The infectious diseases know to be associated with reactive histiocytes, including Whippleís disease, Mycobacterium avium intracellulare infection and malakoplakia, could not be documented. Patients improved under corticosteroid therapy and/or immunomodulatory therapeutic CONCLUSION: A new inflammatory muscle disorder, characterized by a distinctive pathological pattern of macrophagic myofasciitis is emerging in France.

Adult↗
PubMed2000

Bullous pemphigoid and amyotrophic lateral sclerosis: a new clue for understanding the bullous disease?

BACKGROUND: Bullous pemphigoid (BP) occurs in many patients with multiple sclerosis. Isolated cases of BP in patients with other neurological disorders further support a pathogenic association between cutaneous and neurological diseases. Any description of BP in patients with amyotrophic lateral sclerosis is lacking. OBSERVATIONS: We studied a French population of 168 patients with typical amyotrophic lateral sclerosis. Among these, 3 had clinical and histological features of BP. The mean age of the patients was 54 years. None was known to have autoimmune disorders. Results of immunoblot analysis disclosed both anti-BP antigen 1 and anti-BP antigen 2 antibodies. CONCLUSIONS: Bullous pemphigoid seems to be unexpectedly associated with amyotrophic lateral sclerosis. On the basis of the cases presented herein, we discuss the epidemiological significance of the association and the possible interrelation between BP antigen 1 and neurofilaments in the pathogenesis of both disorders.

Aged↗
PubMed2000

Gallium-67 scintigraphy in macrophagic myofasciitis.

OBJECTIVE: To evaluate gallium-67 (67Ga) uptake and the value of 67Ga scintigraphy for diagnosis of macrophagic myofasciitis (MMF), a recently identified inflammatory myopathy. METHODS: Twelve consecutive patients with MMF confirmed by muscle biopsy, 10 with polymyositis, 10 with sarcoidosis, 8 with fibromyalgia, and 10 with lymphoma without muscle symptoms (serving as normal controls for muscle) were included. Patients received 1.8 MBq 67Ga per kg body weight by intravenous injection, and scintigraphy was performed with a 2-head gamma camera. The various views were acquired for the 3 main photopeaks of 67Ga 48 hours after infusion, and were analyzed in 2 blinded experiments by nuclear physicians. A semiquantitative scale was used to compare the uptake of 67Ga in the vascular soft tissue background with that in the muscles or joints of MMF patients, and with that in the normal controls. RESULTS: The MMF patients (4 men and 8 women, mean +/- SD age 47.8 +/- 8.7 years) had chronic myalgia (n = 11; predominantly in the lower limbs), asthenia (n = 10), arthralgia (n = 7), mild muscle weakness (n = 5), and high serum creatine kinase levels (n = 6). All MMF patients had significantly higher levels of 67Ga uptake in the muscle and para-articular areas than that recorded for the soft tissue background and for the controls. Muscle uptake was bilateral, symmetric, and homogeneous, and predominantly localized in the legs. No linear enhancement corresponding to fascias or synovial involvement was observed. In patients with polymyositis, symmetric, but heterogeneous, 67Ga uptake was observed in muscle, but not in the fascia. In patients with sarcoidosis, 67Ga uptake was nodular and heterogeneous in muscle, was not detected in the fascia, and was suggestive of synovial involvement in the joints. The uptake of 67Ga in fibromyalgic patients was similar to that in normal controls and to that in the soft tissue background. CONCLUSION: MMF is a new condition involving characteristic changes that can be detected by deltoid muscle biopsy. It usually manifests as a weakly specific, chronic arthromyalgic syndrome that predominates in the lower limbs. 67Ga scintigraphy is a noninvasive method that may make it easier to differentiate MMF from fibromyalgia and sarcoidosis.

Adult↗
PubMed2000

Brain tuberculomas.

Although brain tuberculomas have become rare in developed countries, diagnosis should be kept in mind when confronted with brain space-occupying lesion(s), particularly in immigrants from endemic countries. Surprisingly, Human Immunodeficiency Virus (HIV) infection does not seem to have affected the incidence of this tuberculous lesion. Clinical, biological and radiological signs are only suggestive. Thus, when no other active extracranial tuberculous process is found, the diagnosis should be confirmed by a biopsy before beginning antituberculous treatment which is rapidly effective. Adjunction of steroids may be warranted owing to the common paradoxical enlargement of lesions during the first weeks of therapy, as is now well described in lymph node tuberculosis. Cure can be obtained in more than 85% of the cases, but neurological sequelae are not rare.

AIDS-Related Opportunistic Infections↗