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Biomedical subjects

P Langevitz

Publications and source records attributed to P Langevitz.

At least 19 recordsLinked to original sources
PubMed2001

A single mutated MEFV allele in Israeli patients suffering from familial Mediterranean fever and Behçet's disease (FMF-BD).

Although familial Mediterranean fever (FMF) is an autosomal recessive disorder, preliminary partial mutation analysis suggested that about 60% of FMF patients, who also suffer from Behçet's disease (FMF-BD), have only a single mutated FMF gene (MEFV). In this study, the possibility that patients with FMF-BD may indeed be carriers of a single mutated MEFV is further analysed. The presence of mutations in the coding region of MEFV of eight patients with FMF-BD, representing six families with 47 members, was determined by sequencing. A possible role for the non-carrier chromosome and for BD in the expression of FMF in patients with a single mutated MEFV allele was determined by analysing the association between these variables and the presence of FMF in heterozygous kin. Sequence analysis revealed that all eight patients had indeed only one mutation in the coding region of MEFV. The patients' non-carrier chromosomes converged into three different MEFV haplotypes and were shared by heterozygous unaffected kin in five of six families. BD was found in 10 of 11 carriers with FMF vs one of 16 carriers without FMF (P < 0.001). These results suggest that FMF may be expressed in individuals harbouring only one coding mutation in MEFV. The findings argue against a role for the non-carrier chromosome in the induction of FMF, and suggest that the FMF phenotype in this cohort was associated with the simultaneous presence of BD. These findings may mirror a more generalised rule, that FMF may be precipitated in carriers of a single mutated FMF gene by factors unrelated to the other MEFV allele.

Alleles↗
PubMed2001

Adverse effects of intravenous immunoglobulin therapy in 56 patients with autoimmune diseases.

OBJECTIVE: To test the adverse effects and viral safety of intravenous immunoglobulin (IVIg) use in autoimmune diseases. METHODS: Fifty-six patients with various autoimmune diseases who were treated with one to six IVIg courses were evaluated for the presence of adverse effects following IVIg therapy and were screened before and after the treatment for the presence of serum human immunodeficiency virus antibodies, hepatitis C virus antibodies, and hepatitis B surface antigen. RESULTS: Among the 56 patients, 20 (36%) had at least one adverse effect following at least one of the treatment courses. These included headache, low-grade fever, chills, anemia, low-back pain, transient hypotension, nausea, intensified perspiration, and superficial and deep vein thromboses. Whereas the presence of adverse effect to IVIg was unrelated to either the clinical response to the treatment or to the nature of the autoimmune disease, the occurrence of an adverse effect in the first treatment course was significantly associated with a greater chance for an adverse effect in the subsequent courses. No transmission of any of the three viral agents examined could be detected. CONCLUSIONS: Although IVIg use in autoimmune diseases is associated with adverse effects in about one third of the patients, these effects are usually mild and transient. Patients who develop adverse effects during the first treatment course may be at increased risk of adverse effects during the subsequent IVIg courses.

Adolescent↗
PubMed2001

Anti-insulin antibodies and the natural autoimmune response in systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is characterized by the finding of ample serum autoantibodies. The role and the origin of many of these antibodies are still obscure. The aim of this work was to study the occurrence of anti-insulin antibodies (AIA) in SLE, and to postulate, based on AIA determination, on the mechanisms involved in the production of some autoantibodies in SLE. IgG and lgM AIA, anti-DNA antibodies (ADA) and anti-tetanus toxoid antibodies (ATA) were determined using ELISA in sera and B-lymphocytes culture media of 24 SLE patients, 10 healthy controls and 19 insulin-dependent diabetes mellitus (IDDM) patients. B- and T-lymphocytes were isolated using Ficoll gradient, depleted of T-cells using cyclosporin A, EBV infected and grown in medium. The frequencies of IgM-AIA and IgG-ADA were higher in SLE patients than in healthy controls (P < 0.02 and P < 0.05, respectively). The rate of IgM-AIA in SLE and IDDM was comparable, while IgG-AIA was significantly less common in SLE than in IDDM (P < 0.05). The prevalence of ATA in SLE patients and healthy controls was similar. These findings increase the spectrum of the humoral autoimmune response in SLE and suggest that part of it (natural autoantibodies) is independent of antigen driven response.

Adolescent↗
PubMed2001

Abdominal CT findings in nephropathic amyloidosis of familial Mediterranean fever.

To evaluate the abdominal CT features of reactive amyloidosis, abdominal CT scans of 20 patients with amyloidosis of familial Mediterranean fever (FMF) were reviewed and compared with abdominal CT scans of 2 control groups: 22 patients with chronic renal failure (CRF) due to non-amyloidotic kidney diseases and 40 patients with normal kidney function. The kidney size of patients with amyloidosis of FMF were found to vary during the course of the disease from normal or slightly larger than normal at the proteinuric phase, to smaller than normal and comparable to kidney size in CRF, at the uremic stage. Compared to kidney disease of other causes, more patients with FMF-amyloidosis had dense kidneys with coarse parenchymal calcification and calcification in other abdominal organs. Patients with FMF-amyloidosis had fewer aortic calcifications than patients with non-amyloidotic kidney disease. These findings suggest that kidney disease of reactive amyloidosis may have abdominal CT findings distinguishing it from other types of kidney diseases.

Abdomen↗
PubMed2001

Prevalence of ischemic heart disease in patients with familial Mediterranean fever.

BACKGROUND: Familial Mediterranean fever is a genetic disorder manifested by recurrent attacks of peritonitis, pleuritis and arthritis, and characterized by clinical, histological and laboratory evidence for localized and systemic inflammation. Colchicine treatment usually prevents the attacks and the associated inflammation. Inflammation may play an important role in the initiation and progression of atherosclerosis and ischemic heart disease. OBJECTIVE: To study the effect of inflammation and its prevention on the occurrence of IHD, using FMF as a model. METHODS AND PATIENTS: We studied the presence of IHD and its risk factors in 290 FMF patients aged 40 years or more, and in two control groups--233 spouses of the FMF patients, and 126 patients with inflammatory diseases obtained from other outpatient clinics, FMF patients were also compared with age and gender-matched individuals from the population reference data of the Israel Ministry of Health. RESULTS: The prevalence of IHD in FMF patients was significantly lower than in the group of controls from other outpatient clinics (15.5% vs. 30.2%, P < 0.05) and comparable with their spouses (11.2%) and with the matched general population in Israel (16%). CONCLUSIONS: These findings suggest that despite the evidence of recurrent inflammation, colchicine-treated FMF patients are not more predisposed to IHD than the normal population.

Adult↗
PubMed2001

[The lack of awareness of the Israeli population regarding gastrointestinal complications from non-steroidal anti-inflammatory drugs].

Non-steroidal anti-inflammatory drugs (NSAID) are potent analgesic agents. They are indicated for rheumatoid arthritis, osteoarthritis, severe acute and prolonged chronic pain. A wide range of analgesic agents are available. The objective of this study was to assess the patient's knowledge of his illness, his knowledge familiarity with NSAID medications and their side effects and thereupon, to evaluate the awareness, utilization, satisfaction and expectations associated with current available treatment. During the year 2000, a randomized telephone screening questionnaire, was completed over a 2 month period for a sample group of the general Israeli population over 50 years of age. Of the 3179 persons contacted, 2028 responded and 292 persons were treated with NSAIDs and further investigated. Fifty percent suffered from chronic musculoskeletal pain, 22% had osteoarthritis, 10% had rheumatoid arthritis, and 18% had undefined chronic pain. The average age of the respondent was 65 years old. The average duration of treatment was 11 years. The most common drugs used by the 292 respondents that were taking NSAIDs were diclofenac (Voltaren)--51% and naproxen (Naxyn)--21%. Only 4% were treated with COX-2 specific inhibitors. Twenty percent of the respondents utilized more than one NSAID simultaneously. Forty seven percent of the patients had peptic ulcer disease but only 39% of them were simultaneously treated with an NSAID and a gastroprotective agent. Sixty eight percent of patients expected amelioration of symptoms while taking NSAIDs. Seventy seven percent of respondents were dissatisfied with treatment. The patients expressed that they did not have a reliable easily accessible source of information on their disorder or on available treatment options. Many respondents were not aware of their medical diagnosis. In general, patients were not aware of all the available NSAIDs or the side effects of gastrointestinal complications. Enhancement of information available to the patient is warranted to improve the provision of efficient therapy, reduce side effects and increase patient satisfaction from therapy.

Aged↗
PubMed2000

Familial Mediterranean fever in two Bedouin families: mutation analysis and disease severity.

Familial Mediterranean fever (FMF) is an autosomal recessive disease prevalent among non-Ashkenazi Jews, Armenians, Arabs, and Turks. The Bedouin are nomad Arab tribes residing in desert margins of the Middle East and Arabia. FMF is quite rare in Bedouins, and here we report on two Bedouin families from southern Israel suffering from this disorder. The MEFV mutations found in the Bedouin patients M694I, V726A, and E148Q are consistent with their Arab origin. The disease severity score showed a mild to moderate severity disease in six patients. The Bedouins, leading a unique nomadic life, may prove instrumental in unraveling the role of environmental factors in the course and severity of FMF.

Amino Acid Substitution↗
PubMed2000

Intravenous immunoglobulin therapy in a patient with lupus serositis and nephritis.

The use of intravenous immunoglobulin (IVIg) has been reported as an immunomodulating agent in several autoimmune diseases, including systemic lupus erythematosus (SLE). Herein we report a SLE patient with severe clinical presentation that included pericarditis, pleural effusion, nephrotic range proteinuria, leukopenia, and lymphopenia. The patient received one course of high-dose IVIg (2.8 g/kg body weight), and within a week of post-IVIg therapy, her condition significantly improved. One-month post-IVIg there were decreased proteinuria, elevated leukocytes and lymphocytes count, decrease in antinuclear and anti-dsDNA antibodies, and disappearance of pericarditis and pleuritis. This case demonstrates the efficacy of IVIg in severe SLE with various clinical manifestations.

Adult↗
PubMed2000

Skin score decrease in systemic sclerosis patients treated with intravenous immunoglobulin--a preliminary report.

The aim of the study was to determine for the first time the response of systemic sclerosis (SSc) patients to treatment with intravenous immunoglobulin (IVIg). Three patients with progressive and rapidly deteriorating disease (mainly affecting the skin) were planned to receive six monthly courses of high-dose IVIg (2 g/kg). All had a thorough physical examination, clinical evaluation by the modified Rodnan total skin thickness score, and measurement of the titres of PM-Scl antibodies before and after the treatment, and before and after each treatment course. Two of the three patients received six IVIg courses as planned and no adverse effects or disease progression occurred during the therapy. The third patient received three courses, after which he developed renal failure and later died of sepsis. All three patients had a large decrease in their skin score after the treatment compared to that before the treatment. No modification of PM-Scl antibody titres was noted in any patient. Intravenous immunoglobulin (IVIg) may have a role in the treatment of SSc patients with rapidly deteriorating skin disease. The specific indications, as well as the safety of this treatment, should be further researched.

Adult↗
PubMed2000

Behçet's disease in Familial Mediterranean fever: characterization of the association between the two diseases.

OBJECTIVES: Familial Mediterranean fever (FMF) is a genetic disease, characterized by attacks of fever and painful manifestations. Several vasculitides are more common in FMF than in the general population. The aim of the study was to define and characterize the association between FMF and Behcet's disease (BD), a form of vasculitis not previously related to FMF. METHODS: We conducted a retrospective study in which FMF patients, also suffering from BD (FMF-BD), were recruited from about 4,000 patients registered in our clinic, using a computer survey. Patients identified by the screening process were examined, and those meeting the published criteria for the diagnoses of FMF and BD were classified as FMF-BD cases and compared with unselected FMF and BD controls. RESULTS: The prevalence of BD was higher in FMF than in populations known to be rich in BD (eg, 16 per 4,000 in FMF compared with 1 per 104 in Japan, P < .001). FMF-BD cases and FMF or BD controls were comparable in most demographic, clinical, and laboratory aspects studied. However, more cases than FMF-controls were of Iraqi/Turkish origin and responded less favorably to colchicine. A higher proportion of cases than BD controls had skin, central nervous system, and gastrointestinal manifestations, originated from North Africa, and had family history of BD. In most cases, as in most respective controls, the severity of FMF was of intermediate grade and the extensiveness of BD was limited. The HLA B5 antigen was present in 53% of BD cases and 40% of BD controls. CONCLUSIONS: BD should be included among the vasculitides complicating FMF. BD and FMF in patients with FMF-BD, and in patients suffering from each of these entities alone, are clinically and demographically comparable.

Adult↗
PubMed2000

Intravenous immunoglobulin treatment of lupus nephritis.

OBJECTIVE: To evaluate the clinical response of treatment-resistant membranous and membranoproliferative lupus nephritis to intravenous immunoglobulin (IVIg). METHODS: Seven lupus nephritis patients who failed to respond to at least prednisone and cyclophosphamide were studied. A kidney biopsy showing either membranous or membranoproliferative glomerulonephritis was available in six patients. They were treated with six courses (patients 1 and 2) or 1 or 2 courses (patients 3 through 7) of high-dose IVIg. For patients 3 through 7, the plasma levels of albumin, total cholesterol, urea, creatinine, dsDNA antibody titers, and daily proteinuria were measured just before the IVIg therapy, immediately on completion, and 6 months later. RESULTS: All seven patients had a beneficial response to IVIg. In patient 1, decrease in proteinuria was evident 2 weeks after IVIg was started, nephrotic syndrome gradually disappeared, and she had no proteinuria in 3 years' follow-up. Decline in proteinuria was evident in patient 2 after the 4th IVIg course, but proteinuria reached the pretreatment level 4 months after the therapy ended. In patients 3 through 7, the mean daily proteinuria before IVIg (5.3 +/- 2.1 g) decreased after 1 or 2 IVIg courses (3.3 +/- 1.4 g), and further decreased when measured 6 months later (2.1 +/- 1.3 g). Similarly, the plasma cholesterol level decreased while the plasma albumin level increased after IVIg. CONCLUSIONS: IVIg might be effective in treatment-resistant membranous or membranoproliferative lupus nephritis. Future studies should concentrate on determining the preferred treatment protocol of IVIg for the various classes of lupus nephritis.

Adolescent↗
PubMed2000

Thromboembolism in a patient with transient eosinophilia and thrombocytopenia.

A 24-year-old woman with an unremarkable medical history who developed bilateral deep venous thrombosis and pulmonary emboli is presented. Associated findings were severe eosinophilia and moderate thrombocytopenia. Since the major acquired and hereditary thrombogenic disorders were ruled out in this case (including antiphospholipid syndrome and heparin-induced thrombocytopenia), we believe that the severe eosinophilia per se could be the pro-coagulant factor leading to thrombosis and embolism in our patient. The role of eosinophilia in thrombosis is discussed.

Adult↗
PubMed2000

Diagnostic and treatment concerns in familial Mediterranean fever.

Familial Mediterranean fever (FMF) is an autosomal, recessively inherited disease, affecting people of Jewish, Arabic, Turkish and Armenian ancestry. The disease is the prototype of the periodic febrile syndromes. Its hallmark is short attacks of fever and painful manifestations in the abdomen, joints, chest, scrotum and skin. Chronic and protracted manifestations, particularly nephropathic amyloidosis, chronic arthritis, and protracted myalgia, may also occur in the disease. The diagnosis of FMF should be considered in individuals of an appropriate ethnic background who present with febrile disease of episodic nature. The differential diagnosis in this case is broad and includes a large number of infectious, inflammatory and genetic diseases. However, in most cases, the very specific general and site-restricted features of the FMF attacks on the one hand, and the absence of manifestations typical of other conditions on the other hand, determine the diagnosis of FMF. This chapter presents clues and tips that help in the diagnosis and treatment of FMF.

Amyloidosis↗
PubMed2000

Erysipelas-like erythema of familial Mediterranean fever: clinicopathologic correlation.

BACKGROUND: Familial Mediterranean fever (FMF) is an autosomal recessive disease that tends to affect certain ethnic groups. It is characterized by recurrent, self-limited attacks of peritonitis, pleuritis, and synovitis. Erysipelas-like erythema (ELE) is the pathognomonic skin manifestation. Lesions are characterized by tender erythematous plaques, usually located on the lower legs. They may be triggered by physical effort and subside spontaneously within 48 to 72 hours of bed rest. Fever and leukocytosis may accompany this condition. OBJECTIVE: The purpose of this study was to describe the histology and the immunofluorescence findings in ELE and to discuss these observations in relation to the clinical findings in FMF. METHODS: We studied 7 patients with FMF in whom ELE developed. In all patients a biopsy was performed within 18 hours from onset of the lesion. In addition to routine hematoxylin and eosin stains, immunohistochemistry to evaluate the infiltrate and direct immunofluorescence were performed. Patients were followed up for their ELE lesions. RESULTS: Histologic examination revealed edema of the superficial dermis and sparse perivascular infiltrate composed of a few lymphocytes, neutrophils, and nuclear dust. Vasculitis was not observed. Direct immunofluorescence showed, in all cases, deposits of C3 in the wall of the small vessels of the superficial vascular plexus. In some cases fibrinogen and IgM were also observed. CONCLUSION: These findings are in accordance both with those found previously in the erysipelas-like phenomenon and those in the peritoneum of patients with FMF. The sparse infiltrate and the deposition of C3 also are compatible with the clinical picture of self-resolving lesions of short duration. It also suggests that erysipelas-like erythema belongs to the spectrum of neutrophilic dermatoses and supports a pathogenesis that involves abnormal inhibition of the inflammatory cascade.

Adult↗
PubMed2000

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Journal Article↗
PubMed2000

Protracted febrile myalgia of familial Mediterranean fever. Mutation analysis and clinical correlations.

Protracted febrile myalgia (PFM) is a rare form of vasculitic disease that affects patients with familial Mediterranean fever (FMF). Mutation analysis performed in 15 patients who suffered from this disorder showed that 9 of the 15 patients were homozygous for M694V. FMF in these 9 patients was associated with more severe symptoms compared to a group of 30 M694V homozygous FMF patients without PFM.

Adolescent↗