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Biomedical subjects

P Langevitz

Publications and source records attributed to P Langevitz.

At least 73 records · Page 4Linked to original sources

High incidence of malignancies in patients with dermatomyositis and polymyositis: an 11-year analysis.

OBJECTIVES: Dermatomyositis and polymyositis (DM/PM) are associated with neoplasms. The aim of the present study is to compare our experience in Israel with other published data. METHODS: Thirty-five adult patients with DM/PM, admitted to Sheba Medical Center during the 11-year interval between 1984 and 1994, were studied for the prevalence and features of malignant diseases. Patients with DM/PM alone and with DM/PM and malignancy were identified by using the hospital computer system. The manifestations of DM/PM and features of the malignant diseases were abstracted from the patients' charts. The presence or absence of malignancy and the type of cancer were verified in the National Cancer Registry. RESULTS: There were 15 men and 20 women. The mean age at the onset of the disease was 53 +/- 18 years. A total of 15 had PM and 20 DM. Malignancies occurred in four patients with PM (27%) and in nine with DM (45%) a frequency 12.6 times higher than in the general population. In six patients, the malignancy and the DM/PM were diagnosed simultaneously; in four before and in three after the appearance of the DM/PM. Hematologic, gastrointestinal, breast, ovarian, and lung tumors, malignant melanoma, and metastatic carcinoma of unknown primary were found among our patients. Eight DM/PM patients with malignancy died during the study period of infection, pulmonary embolism, and tumor spread. CONCLUSIONS: Our study found that DM/PM is associated with high rates of malignancy and mortality.

Adult↗

Calcification in untreated non-Hodgkin's mediastinal lymphoma.

We report a case of calcifications in mediastinal non-Hodgkin's lymphoma. Although calcification may occur in lymphoma after chemotherapy or radiotherapy in areas of fibrous healing and scar formation, it has been reported only rarely in untreated non-Hodgkin's lymphoma.

Adult↗

Immunomodulation of murine experimental SLE-like disease by interferon-gamma.

The objective of this study was to assess the impact of murine recombinant IFN-gamma and anti-IFN-gamma monoclonal antibody on the BALB/c mice experimental model of lupus. BALB/c female mice were immunized with a human anti-DNA antibody that carries the 16/6 idiotype. These mice were divided into several therapeutic groups according to different treatment strategies; injection with mouse recombinant IFN-gamma, anti-IFN-gamma mAb, phosphate-buffered saline (PBS), irrelevant mouse IgG and control groups that were neither treated nor immunized with the human anti-DNA antibody. The administration of IFN-gamma, intensified the degree of clinical, histological and serological parameters in this model of BALB/c murine lupus. This immunomanipulation decreased the mice longevity. All the laboratory parameters reflected acceleration of the disease in the IFN-gamma treated group as an elevated sedimentation rate, decreased white blood cell count and the development of massive proteinuria. One month after the boost injection, all the mice that were immunized with the anti-DNA antibody, developed high titers of autoantibodies; however, following an additional month, their levels declined in the IFN-gamma treated group. These findings were in concordance with an increased glomerular deposition of immune complexes in the IFN-gamma treated mice. IFN-gamma upregulated the levels of IL-4 and increased the number of IL-4 and IL-6 secreting splenocytes. In conclusion IFN-gamma administration can aggravate the clinical and laboratory outcome of 16/6 id induced lupus in BALB/c mice.

Animals↗

Criteria for the diagnosis of familial Mediterranean fever.

OBJECTIVE: To establish a new set of criteria for the diagnosis of familial Mediterranean fever (FMF). METHODS: Twenty-seven features and manifestations typical of FMF were studied to determine their prevalence in 105 patients with FMF and 106 controls. Diagnosis of FMF in the study group was based on clinical judgment. Controls were patients with a variety of other diseases who presented to the emergency room or outpatient clinics with recurrent episodes of pain in body sites usually involved in FMF attacks. Manifestations observed to be significantly more common in FMF patients than in controls were incorporated into the rule proposed for diagnosis of FMF, based on a model of major, minor, and supportive criteria. RESULTS: Two sets of diagnostic criteria were established. A conservative criteria set for diagnosis of FMF was based on the presence of 1 major or 2 minor criteria, or 1 minor plus 5 supportive criteria, and a simple criteria set for diagnosis of FMF required 1 major or 2 minor criteria. The sensitivity and specificity of these 2 criteria sets were >95% and >97%, respectively. CONCLUSION: The proposed new sets of criteria were highly sensitive and specific, and could be used to readily diagnose FMF and to distinguish FMF from other periodic febrile diseases.

Adolescent↗

Antiperinuclear factor--clinical, serological and genetic correlates in Israeli patients with rheumatoid arthritis.

The possible association between the presence of antiperinuclear factor (APF) and clinical and genetic parameters was investigated in 54 Israeli patients with rheumatoid arthritis (RA). Rheumatoid factor (RF) was detected in the sera of 43 patients (80%) and APF was positive in 33 (61%). No significant statistical differences were found in the presence of HLA-DR4 and/or DR1 between APF-positive and -negative patients. Furthermore, neither the Ritchie articular index nor the patient's functional class correlated with the presence of APF. The results of our study suggested that although Israeli patients have a different genetic background, the presence and behaviour of APF is similar to that of other Caucasian populations.

Adult↗

Seronegative spondyloarthropathy in familial Mediterranean fever.

To define a possible association between familial Mediterranean fever (FMF) and seronegative spondyloarthropathy (SNSA) and to study features of SNSA in FMF patients, we screened for the presence and manifestations of SNSA in 3,000 FMF patients attending the National Center for FMF in our institution. This population included 160 patients with chronic arthritis, most who suffered from SNSA. Patients were considered to suffer from SNSA if they had chronic arthritis, inflammatory back/neck pain, and sacroiliitis. Patients who had other diseases associated with SNSA were excluded. Eleven patients, nine men and two women, with chronic monoarthritis or oligoarthritis, grade 2 (four patients) or grades 3 to 4 (seven patients), sacroiliitis, and inflammatory back pain met the criteria for diagnosis of SNSA of FMF. These patients were rheumatoid factor (RF) and HLA-B27 negative. In seven patients, spondyloarthropathy developed while they received colchicine, and in four before colchicine. Most patients responded to treatment with nonsteroidal antiinflammatory drugs, but three required second-line agents. These findings suggest that SNSA is one of the musculoskeletal manifestations of FMF that may occur despite colchicine therapy and requires specific treatment.

Achilles Tendon↗

Chronic massive knee effusion in familial Mediterranean fever.

OBJECTIVE: To document the occurrence of chronic massive knee effusions in patients with familial Mediterranean fever (FMF). PATIENTS: Three cases of chronic massive knee effusion of young FMF patients requiring open synovectomy are presented. RESULTS: Open synovectomy necessitated protracted rehabilitation, eventually with good results. CONCLUSIONS: FMF should be considered in the differential diagnosis of chronic massive knee effusion. Early diagnosis and daily colchicine treatment generally result in a good clinical outcome and may eliminate the need for open synovectomy in favor of arthroscopic surgery.

Adult↗

Clinically insignificant (natural) autoantibodies against acetyl cholinesterase in the sera of patients with a variety of neurologic, muscular and autoimmune diseases.

Acetyl cholinesterase (AChE) antibodies were shown to be associated with myasthenia-like neuromuscular disease. However, it is not clear whether they cause the disease, or their presence is secondary to the disease or an unrelated epiphenomenon. Therefore, AChE antibodies were studied in the sera of 135 patients with neurologic, muscular and autoimmune diseases, using enzyme linked immunosorbent assay (ELISA), immunoblotting and enzyme inhibition assay. In 12 sera the AChE binding by ELISA was greater than 2 standard deviations (SDs) above the mean value of the 20 healthy controls. However, this increased binding was not disease-specific, had no clinical correlates and could not be demonstrated using Western blotting and AChE enzyme inhibition assay, suggesting that these antibodies are naturally occurring, pathogenically unimportant autoantibodies. The finding also supports a possible pathogenic role for the previously reported, high titer, high affinity, inhibitory AChE antibodies in the neuromuscular disease.

Acetylcholinesterase↗

Attacks of pericarditis as a manifestation of familial Mediterranean fever (FMF).

Familial Mediterranean fever (FMF) is characterized by recurrent attacks of febrile serositis. While arthritis, pleuritis and peritonitis are common in FMF, no association of pericarditis with FMF has been described in detail. We retrospectively studied about 4000 FMF patients, using a computer chart review. Pericarditis was diagnosed when patients sustained attacks of pleuritic retrosternal chest pain and had typical findings in the electrocardiogram, echocardiogram or chest radiogram. The incidence and features of pericarditis in FMF were compared to published data. Over a period of 20 years, one or more episodes of pericarditis were recorded in 27 patients, a significantly higher incidence than in the general population (68 vs. 6 per 10(5) per year, p < 0.001). Each patient experienced 1-3 pericarditis attacks, lasting a mean of 4.2 days, accompanied by high temperature and symptoms of FMF attack at another site. The pericarditis attack resolved spontaneously and left no sequelae. FMF patients with pericarditis were comparable to other FMF patients in most demographic and clinical parameters. Pericarditis may be considered another rare manifestation of FMF.

Adolescent↗

Lymphocytotoxic antibodies in Israeli patients with rheumatoid arthritis.

The presence, antigenic specificity, and clinical role of lymphocytotoxic antibodies (LCAs) were studied in 72 Israeli patients with rheumatoid arthritis (RA). Using the microlymphocytotoxicity assay on a cell panel of 47 donors, LCAs were found in 55% of the 72 RA sera, each displaying a distinct pattern of anti-lymphocytic reactivity, mostly against B lymphocytes. Human lymphocytic antigens (HLA) analysis of donors' lymphocytes suggested that activity of LCA-positive RA sera is HLA directed in 60% of cytotoxic sera. The anti-HLA antibodies found were not autoreactive and were not restricted to a particular class I or class II antigen. Relating the presence of LCAs to selective clinical features revealed that LCAs are inversely associated with the presence of an erosive disease (P <0.01) and with the patients' HLA-DQw1 (P <0.01). These findings suggest that LCAs in Israeli patients with RA are very common, multispecific and may have a protective role not mediated through interaction with self-HLA antigens.

Antilymphocyte Serum↗

Familial Mediterranean fever and hyperimmunoglobulinemia D syndrome: two diseases with distinct clinical, serologic, and genetic features.

OBJECTIVE: To determine whether the 2 periodic febrile syndromes familial Mediterranean fever (FMF) and hyperimmunoglobulinemia D syndrome (HIDS) are distinct diseases. METHODS: Clinical manifestations of the diseases were analyzed by physicians experienced with FMF and HIDS. Serum immunoglobulin (Ig) levels were studied in 70 patients with FMF using nephelometry or ELISA and compared with Ig levels in 50 patients with HIDS. Genetic linkage of HIDS with the chromosome 16 polymorphic locus RT70, currently used for refined localization of the FMF susceptibility gene (MEFV), was studied in 9 HIDS families (18 patients) using polymerase chain reaction amplification and gel electrophoresis. RESULTS: The main clinical features distinguishing FMF from HIDS were lymphadenectomy, skin eruption, and symmetrical oligoarthritis in HIDS, and monoarthritis, peritonitis, and pleuritis in FMF. Increased IgG levels were found in 12 patients with FMF (17%), IgA in 16 (23%), IgM in 9 (13%), and IgD in 9 (13%), significantly lower than the prevalence reported for HIDS. We found no evidence for genetic linkage between HIDS and the chromosome 16 marker RT70. CONCLUSION: HIDS and FMF are different entities, clinically, immunologically, and genetically.

Chromosomes, Human, Pair 16↗

Low-molecular-weight heparin during pregnancy and delivery: preliminary experience with 41 pregnancies.

OBJECTIVE: To describe experience with 41 pregnancies treated with the low-molecular-weight heparin enoxaparin. METHODS: The medical charts of 34 women (a total of 41 pregnancies) treated between January 1992 and March 1995 with the low-molecular-weight heparin enoxaparin were reviewed. Most patients (87.5%) received one daily 40-mg injection. In all cases, treatment was continued throughout labor, delivery, and the immediate postpartum period. RESULTS: Therapy was administered for 5-280 days (median 91). One case of a thromboembolic event was recorded during treatment. No systemic or local side effects were reported. During pregnancy, only one patient had mild vaginal bleeding, which resolved spontaneously while therapy was continued. There was no excessive intrapartum bleeding in any of these patients, whether delivered vaginally or abdominally. During treatment, 19 of the 34 patients underwent 24 surgical procedures, including 13 cesarean deliveries, without excessive bleeding. Epidural anesthesia was used during labor in nine of the patients, with no specific complications. The corrected perinatal mortality rate, (ie, the rate of fetal death after 24 weeks' gestation plus neonatal death, excluding a neonate with multiple anomalies) for those neonates delivered after 24 weeks' gestation was 2.7%. There were no cases of intraventricular hemorrhage in any of the neonates. CONCLUSION: This preliminary series, the largest reported to date, demonstrates the relative safety and efficacy of low-molecular-weight heparin therapy in pregnancy and delivery.

Adult↗

The changing face of familial Mediterranean fever.

Familial Mediterranean fever (FMF) is a genetic disease characterized by painful febrile "attacks" of serositis and the development of amyloidosis. Although FMF has been extensively studied and described, new data have accumulated during the last decade. This report gives an update, focusing specifically on (1) newly characterized manifestations, such as acute scrotal "attacks," protracted febrile myalgia, and spondyloarthropathy; (2) progress made in the diagnosis and treatment of FMF-amyloidosis; (3) experience acquired with colchicine, establishing its safety in common practice, childhood, conception, and pregnancy; (4) colchicine's role in the prevention and treatment of FMF-amyloidosis; (5) new laboratory findings; and (6) new considerations in the differential diagnosis. The most important achievement in recent years, however, is the mapping of the FMF susceptibility gene to chromosome 16p, a finding that raises hopes for prompt cloning of the gene and elucidation of the mechanisms involved in FMF expression.

Cohort Studies↗

Aspergillosis in systemic lupus erythematosus.

Infection is the major cause of morbidity and mortality in systemic lupus erythematosus (SLE). Although various fungi account for a substantial number of these lethal infections, aspergillosis, an important opportunistic infection in immunosuppressed patients, is described rarely. Only 23 cases have been reported in the English-language medical literature. Risk factors for acquiring aspergillosis in these patients were high grade disease activity, granulocytopenia, use of steroids and other immunosuppressive treatment and presence of bacterial infection. The diagnosis in most patients was delayed and they died. Here, we describe three SLE patients with invasive aspergillosis. Features of our patients' diseases were similar to those reported previously. Aspergillosis appeared while they had active SLE treated with high dose corticosteroids. In 2 patients the fungal infection was systemic and diagnosed post mortem. Both were leukopenic and had concurrent bacterial infection and one received amphotericin B prior to death. In the third, the infection was localized to a transplanted kidney and was cured by nephrectomy. Aspergillosis should be suspected in patients with active SLE, who are immunocompromised and sustain concomitant bacterial infections. The currently poor prognosis may be improved with more aggressive diagnostic investigation and treatment.

Adolescent↗