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P Langevitz

Publications and source records attributed to P Langevitz.

At least 37 records · Page 2Linked to original sources
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↗
PubMed2000

Dermatomyositis and polymyositis associated with the antiphospholipid syndrome-a novel overlap syndrome.

OBJECTIVE: As APS (antiphospholipid syndrome) can be either primary or secondary to a wide range of other conditions (such as autoimmune diseases, malignancies, infectious diseases, and drug-induced conditions) the aim of this study was to describe a novel overlap syndrome of APS. METHODS: All patients diagnosed with either PM (polymytosis) or DM (dermatomytosis) who were treated in the Rheumatology Unit, Sheba Medical Center, were followed-up in the past 8 years for the appearance of a clinical manifestation of the APS, and conversely, patients with APS treated by us were clinically assessed for the presence of signs and symptoms of PM and DM. Both conditions were diagnosed according to accepted diagnostic criteria. RESULTS: Three patients were found to have both APS and PM/DM. A patient with PM had transverse myelopathy, a patient with DM had pulmonary embolism, and a patient with PM had recurrent abortions, stroke, livedo reticularis and mitral regurgitation. Both patients with PM had also SLE (systemic lupus erythematosis). CONCLUSIONS: APS can be associated with a wide range of diseases. Future data would reveal which therapy is the best for the association of PM/DM with APS, and determine the pathogenesis and prognosis in patients with this association.

Adult↗
PubMed2000

Benefits and risks of minocycline in rheumatoid arthritis.

Rheumatoid arthritis is a chronic inflammatory disease affecting about 1% of the adult population. The pathophysiology of rheumatoid arthritis remains incompletely understood. An infectious aetiology of the disease has long been postulated, but not proved. Despite insufficient evidence for the infectious nature of this disorder, several antibacterials, such as sulfa compounds, tetracyclines and rifampicin, have been investigated in the treatment of rheumatoid arthritis. In the last few years, minocycline, a semi-synthetic derivative of tetracycline, has been extensively studied as a therapeutic agent for rheumatoid arthritis. The antirheumatic effect of minocycline can be related to its immunomodulatory and anti-inflammatory, rather than to its antibacterial properties. Its efficacy in rheumatoid arthritis has been reported in 2 open trials and in 3 double-blind controlled studies. The first 2 double-blind studies, 1 in The Netherlands and 1 in the US, were performed in patients with advanced disease. Both studies showed a modest, but statistically significant improvement in the clinical parameters of disease activity and in the erythrocyte sedimentation rate in the minocycline-treated patients. The US study also reported that patients in the minocycline group developed fewer erosions than those in the placebo group. This finding supports the role of minocycline as a disease modifying agent. The common adverse effects of minocycline reported in these 2 studies included gastrointestinal adverse effects, dizziness, rash and headaches. Less common adverse effects were intracranial hypertension, pneumonitis, persistent skin and mucosal hyperpigmentation, lupus-like syndrome and acute hepatic injury. The third double-blind study enrolled only seropositive rheumatoid arthritis patients with early disease (less than 1 year duration), and showed very encouraging results of significant improvement in the disease activity parameters in the minocycline treated group of patients. The same authors later reported that about half of these patients were in or near remission after 3 years of follow up. No adverse effects were reported in this study. Summarising the data of these 3 double-blind studies, we may conclude that minocycline may be beneficial in patients with rheumatoid arthritis, especially when given early in the disease course or in patients with a mild disease.

Animals↗
PubMed2000

Genotype-phenotype assessment of common genotypes among patients with familial Mediterranean fever.

OBJECTIVE: To study genotype-phenotype correlation for the 4 most common genotypes found among patients with familial Mediterranean fever (FMF). METHODS: Thirty patients with the M694V/M694V genotype, 32 with M694V/V726A genotype, 25 with M694V/E 148Q genotype, and 21 with V726A/V726A genotype were assessed for various clinical manifestations of FMF, and overall disease severity. RESULTS: Patients with the M694V/M694V genotype were found to have an earlier age of onset, higher frequency of joint involvement, higher frequency of erysipelas-like erythema, and required higher doses of colchicine to control the disease compared to the other 3 genotypes. CONCLUSION: The M694V/M694V genotype is associated with more severe disease compared to other common genotypes in patients with FMF.

Age of Onset↗
PubMed1999

Late-onset familial Mediterranean fever (FMF): a subset with distinct clinical, demographic, and molecular genetic characteristics.

To determine the prevalence and characterize demographic, clinical, and genetic features of familial Mediterranean fever (FMF) of late onset, all patients experiencing their first FMF attack at age 40 years or more were identified using the computerized registry of our FMF clinic, and then thoroughly interviewed and examined. The control group consisted of 40 consecutive FMF patients, who arrived at the FMF clinic for their regular follow-up visit and were 40 years of age or older at the time of the examination. The severity of the disease in patients and controls was determined using a modified score, developed previously. Mutational analysis in the FMF gene was performed using a commercial kit. Only 20 of 4000 (0.5%) patients had late-onset FMF. These patients were mostly men, of non-North African origin, P < 0.05 compared to controls. All had abdominal attacks and in most these were the only manifestation of their disease, P < 0.001. None had chronic or prolonged manifestations of FMF, for example, amyloidosis, chronic arthritis, or protracted myalgia, P < 0.001. The response to treatment was good despite using low colchicine dose, P < 0.05. The overall severity score indicated a mild disease, P < 0.001. Mutational analysis revealed absence of M694V homozygosity, P < 0.01, compared to our regular FMF population. We conclude that the onset of FMF in a late age defines a milder form of disease with typical clinical, demographic, and molecular genetic characteristics.

Adult↗
PubMed1999

Monoclonal anti-endothelial cell antibodies from a patient with Takayasu arteritis activate endothelial cells from large vessels.

OBJECTIVE: To create monoclonal anti-endothelial cell antibodies (mAECA) from a patient with Takayasu arteritis to evaluate their ability to activate human umbilical vein endothelial cells (HUVEC), and to characterize the mechanism of EC activation. METHODS: A panel of mAECA was generated from peripheral blood lymphocytes of a patient with Takayasu arteritis, using Epstein-Barr virus transformation. Activity against macrovascular EC (HUVEC) and microvascular EC (human bone marrow EC immortalized by SV40) antigens was detected by enzyme-linked immunosorbent assay. Inhibition studies were used to select the monoclonal antibodies (mAECA) which share the same EC epitope binding specificity as the total IgG-AECA from the Takayasu arteritis patient. The binding of the mAECA to human aortic EC was studied by immunohistochemistry. The secretion levels of interleukin-6 (IL-6) and von Willebrand factor (vWF) were determined, to serve as markers for EC activation. The activated EC were examined for the adherence of a monocytic cell line (U937), as well as for expression of vascular cell adhesion molecule 1, intercellular adhesion molecule 1, and E-selectin. In addition, nuclear extracts of the mAECA-treated EC were analyzed for the induction of translocation of nuclear factor kappaB (NF-kappaB), using a specific NF-kappaB oligoprobe in an electrophoretic mobility shift assay. RESULTS: Six mAECA were selected, the mixture of which produced 100% inhibition of binding of the original IgG (from the patient with Takayasu arteritis) to HUVEC. All mAECA possessed high activity against macrovascular EC, but none had significant antimicrovascular EC activity. The mAECA, but not normal human IgG, had anti-human aortic EC activity. Four of the 6 mAECA activated EC, manifested by increased IL-6 and vWF secretion. The 4 mAECA induced EC expression of adhesion molecules and increased adhesion of U937 monocytic cells to EC. In addition, these mAECA stimulated the nuclear translocation of the NF-kappaB transcription factor. CONCLUSION: Our findings suggest that AECA may directly stimulate EC in Takayasu arteritis through elevation of adhesion molecule expression associated with NF-kappaB activation and adhesion of monocytes, and may therefore play a pathogenic role in the development of the vasculopathy in Takayasu arteritis.

Adult↗
PubMed1999

Successful treatment of systemic lupus erythematosus cerebritis with intravenous immunoglobulin.

Neuropsychiatric lupus includes extremely diverse clinical manifestations, ranging from mild cognitive dysfunction to a severe, life-threatening presentation. We report a 28-year-old patient with systemic lupus erythematosus who had persistent fever for 3 months, and developed within a few hours motor and sensory aphasia, rotator nystagmus with deviation of the eyes, and severe nuchal rigidity. An extensive series of imaging and laboratory tests were interpreted as normal, except for an elevated opening pressure at lumbar puncture, cerebrospinal fluid inflammatory findings, and asymmetrical cortical perfusion on single-photon emission computed tomography. The patient received one course of high-dose intravenous immunoglobulin (IVIg) and within 5 days her condition returned to that of 3 months before admission. The mechanisms of injury, along with the management of cerebral lupus and the mechanisms of action of IVIg, are discussed.

Adult↗
PubMed1999

Periodic fever, aphthous stomatitis, pharyngitis, and adenopathy syndrome: clinical characteristics and outcome.

We report 28 patients (20 male) with a syndrome characterized by abrupt onset of fever, malaise, aphthous stomatitis, tonsillitis, pharyngitis, and cervical adenopathy (PFAPA syndrome). Episodes of fever occurred at intervals of 5.1 +/- 1.3 weeks beginning at the age of 4.2 +/- 2.7 years. Fever, malaise, tonsillitis with negative throat cultures, and cervical adenopathy were reported in all 28 patients, aphthae in 19, headache in 5, abdominal pain in 5, and arthralgia in 3. Mild hepatosplenomegaly was observed in 6 patients. Mild leukocytosis, elevation of the erythrocyte sedimentation rate, and fibrinogen were found during attacks. These episodes of illness resolved spontaneously in 4.3 +/- 1.7 days. Serum IgD was found elevated (>100 U/mL) in 12 of the 18 patients tested (140.2 +/- 62.4 U/mL). Affected children grow normally, have no associated diseases, and have no long-term sequelae. Attacks were aborted by a single dose of oral prednisone (2 mg/kg) at the beginning of the attack in all 15 patients in whom this medication was prescribed. In 9 patients the syndrome has completely resolved (beginning at the age of 2.9 +/- 1.3 and lasting 8 +/- 2.5 years). In 3 other patients complete resolution of the attacks occurred after tonsillectomy was performed. PFAPA is sporadic, and no ethnic predilection was found. Increased awareness of the clinical syndrome has resulted in more frequent diagnosis and adequate treatment.

Age of Onset↗