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Biomedical subjects

P Langevitz

Publications and source records attributed to P Langevitz.

At least 91 records · Page 5Linked to original sources
PubMed1996

Cyclosporine therapy for acquired factor VIII inhibitor in a patient with systemic lupus erythematosus.

The case of a 27-year-old woman with systemic lupus erythematosus and development of an autoantibody against factor VIII during an exacerbation of her underlying disorder is described. Attempts to eliminate the antibody with high dose gammaglobulin and repeated courses of cyclophosphamide failed, whereafter she received cyclosporine in increasing doses. When therapeutic serum levels of cyclosporine were achieved (150-350 ng/ml) the inhibitor rapidly decreased and disappeared with a concomitant normalization of the factor VIII levels. Treatment with cyclosporine was subsequently reduced and discontinued after one year, and at present no inhibitor is detectable. In view of the successful results with cyclosporine treatment in 4 of 6 previous cases and in all 3 previous cases with autoimmune disorders, this regimen should be evaluated in a systematic manner as a potential first line drug in patients with acquired hemophilia and an underlying autoimmune disorder.

Adult↗
PubMed1994

[Atrial septal aneurysm and paroxysmal atrial fibrillation].

A 48-year-old man was admitted because of treatment-resistant, paroxysmal atrial fibrillation. Transthoracic, 2-dimensional echocardiography revealed a "mass" within the left atrium, thought to be a tumor. Transesophageal echocardiography showed it to be an atrial septal aneurysm. There is a relationship between atrial septal aneurysm and atrial supraventricular tachyarrhythmias and systemic emboli.

Atrial Fibrillation↗
PubMed1994

Colchicine treatment of AA amyloidosis of familial Mediterranean fever. An analysis of factors affecting outcome.

OBJECTIVE: To elucidate factors possibly influencing the outcome of colchicine therapy in patients with amyloidosis of familial Mediterranean fever (FMF). METHODS: Retrospective analysis of data abstracted from the charts of all 68 FMF patients with amyloidosis who presented during the study period (1974-1992) with proteinuria (> or = 0.5 gm/24 hours) and creatinine values < or = 2.5 mg/dl, received colchicine, and were followed up for > or = 5 years. RESULTS: At the end of the study period, kidney disease had worsened in 31 patients and remained stable in 22. Proteinuria had regressed in 15 patients. Deterioration was related to initial serum creatinine values > or = 1.5 mg/dl (P < 0.01) and to mean colchicine dosage < or = 1.5 mg/day (P < 0.001). The 3 groups were comparable in terms of initial urinary protein levels, duration of proteinuria, presence of hypertension, occurrence of febrile attacks, sex distribution, and proportion of non-compliant patients. CONCLUSION: The therapeutic dosage of colchicine for amyloidosis of FMF is > 1.5 mg/day. This dosage is effective only in patients with initial serum creatinine levels < 1.5 mg/dl.

Adult↗
PubMed1994

Recurrent episodes of acute scrotum with ischemic testicular necrosis in a patient with familial Mediterranean fever.

The tunica vaginalis is 1 of the sites involved in the recurrent febrile attacks of serositis, which are the hallmark of familial Mediterranean fever. The attacks present clinically as "orchitis." We report on a patient with familial Mediterranean fever in whom recurrent episodes of scrotal attacks were complicated by testicular necrosis requiring orchiectomy. The case emphasizes the challenge of recognizing and differentiating these attacks from other causes of acute scrotum.

Acute Disease↗
PubMed1994

Prevention of recurrent aphthous stomatitis with colchicine: an open trial.

BACKGROUND: Recurrent aphthous stomatitis (RAS) is a common disorder with hitherto unsatisfactory drug therapy. OBJECTIVE: Our purpose was to evaluate the prophylactic effect of colchicine in the treatment of RAS. METHODS: An open, prospective, 4-month study was conducted in 20 patients with RAS who served as their own controls. During the first 2 months of the study no medications were given and in the last 2 months colchicine, 1.5 mg/day, was prescribed. RESULTS: The mean number of aphthae per week and the subjective daily pain scores were reduced by 71% and 77%, respectively, during colchicine treatment as compared with the previous period (p < 0.001 for both). No serious side effects of colchicine were noted. CONCLUSION: These findings suggest a role for continuous colchicine therapy in the prevention of RAS.

Adolescent↗
PubMed1994

Protracted febrile myalgia in patients with familial Mediterranean fever.

OBJECTIVE: We describe a newly defined syndrome of protracted febrile myalgia in patients with familial Mediterranean fever (FMF). METHODS: Fourteen patients with FMF were admitted with an attack of severe disabling myalgia accompanied by fever, high erythrocyte sedimentation rate, and hyperglobulinemia, lasting up to 6 weeks. RESULTS: Unlike in the classical manifestations of FMF response to corticosteroids therapy was prompt. CONCLUSION: Protracted febrile myalgia is an uncommon dramatic manifestation of FMF that may occur despite colchicine therapy and requires treatment with corticosteroids.

Adolescent↗
PubMed1994

Fibromyalgia in familial Mediterranean fever.

OBJECTIVE: To determine whether chronic lower body pain in a subpopulation of patients with familial Mediterranean fever (FMF) is due directly to the musculoskeletal manifestations of FMF or whether they are connected to mechanical problems in the low back and leg/foot or to other factors operative in fibromyalgia (FM). METHODS: In 93 consecutive patients with FMF a point count of 14 tender points (TP) was conducted by thumb palpation. Tenderness thresholds were assessed in some of the TP and of control point sites by Chatillon dolorimeter. RESULTS: In female patients with FMF dolorimeter thresholds of fibrositic and control point sites were significantly lower than in male patients with FMF (p < 0.004). Also patients with FMF with back pain and foot/leg pain are more tender than patients with FMF without this characteristic (p < 0.001). CONCLUSION: The detection of FM and definition of tenderness thresholds is relevant to this disease, since musculoskeletal complaints are common in this group of patients but not always explained by objective findings.

Adolescent↗
PubMed1993

Colchicine in the treatment of AA and AL amyloidosis.

Colchicine is an effective medication in the prevention and treatment of amyloidosis of familial Mediterranean fever. Its therapeutic effect depends on the stage of renal disease and the drug dose. To evaluate colchicine effect in AA amyloidosis of other diseases and in primary AL amyloidosis, the literature was reviewed. Findings were that (1) the effect of colchicine in reactive amyloidosis has not been methodically studied, but anecdotal reports suggest it may be beneficial; and (2) the results of studies and case reports on the effect of colchicine in primary amyloidosis are conflicting. Because a therapeutic effect of colchicine in primary and reactive amyloidosis has been shown in sporadic cases, a prospective, controlled, multicenter study assessing the effect of colchicine in all types of amyloidosis appears to be justified. Until such a study is available, the addition of colchicine in an appropriate dose to any therapeutic regimen of patients with AA or AL amyloidosis should be considered.

Adult↗
PubMed1993

Total hip replacement in familial Mediterranean fever.

Familial Mediterranean fever (FMF) is a hereditary disorder affecting people of Mediterranean stock, mainly Sephardic Jews and Armenians. It is characterized by attacks of arthritis, either short, self-limited episodes typically lasting 72 hours or protracted attacks lasting from two weeks to one year. The latter form affects mainly the large joints of the lower limb. The hip joint is the most vulnerable and likely to be affected by the protracted attacks, which may result in destruction of the articular cartilage and, in some cases, aseptic necrosis of the femoral head. Eighteen FMF patients (19-52 years) underwent 22 total hip replacements between 1971 and 1985 at our hospital. Six of the 18 initial prostheses experienced aseptic loosening. This relatively high incidence led us to recommend implantation of cementless hip prostheses following meticulous synovectomy as the treatment of choice. The results of these surgeries and the uniqueness of total hip replacement in FMF patients are presented here and discussed.

Adult↗
PubMed1993

Differences in the expression of spondyloarthropathy: a comparison between ankylosing spondylitis and psoriatic arthritis.

The aim of the study was to compare the spondyloarthropathy of ankylosing spondylitis (AS) to that of psoriatic arthritis (PsA). Forty patients with AS and 66 patients with PsA underwent a complete assessment according to a standard protocol, including radiographic evaluation and HLA typing. A logistic regression analysis was performed controlling for age, arthritis duration, and sex. A higher frequency of inflammatory neck and back pain and stiffness (p < 0.0002), limitation of back movements (p < 0.0006), grade 4 sacroiliitis (p = 0.007), and syndesmophytes (p = 0.005) was noted in AS, while peripheral arthritis was more common and more severe in patients with PsA (p = 0.002). A lower frequency of HLA B17 (p = 0.02), and a higher frequency of HLA B27 (p = 0.0001), and Cw2 (p < 0.01) was found in AS compared to PsA. Thus, there are clinical, radiologic, and genetic differences in disease expression of the SA of AS and PsA, supporting their classification as distinct entities.

Adolescent↗
PubMed1992

HLA alleles in systemic sclerosis: association with pulmonary hypertension and outcome.

HLA antigen distribution was studied in 126 patients with systemic sclerosis (SSc) followed prospectively and compared to that of 325 healthy controls. The frequencies of HLA antigens DR3, DR5 and DRw52 were increased in patients with diffuse skin involvement (P = 0.02, 0.05, 0.03). The presence of DRw52 (relative risk [RR] much much greater than 1) and DRw6 (RR = 54.5) was associated with significantly increased risks of a fatal disease outcome with pulmonary hypertension (PHT). In the absence of PHT, DRw252 was inversely associated with the risk of death. These findings indicate an adverse prognosis in SSc when PHT is present in association with DRw52.

HLA Antigens↗
PubMed1992

Treatment of resistant rheumatoid arthritis with minocycline: an open study.

In a 48-week open trial, 18 patients with active rheumatoid arthritis (RA), resistant to second line agents, received 200 mg minocycline daily. Twelve patients completed 48 weeks of therapy. Statistically significant improvement was noted in almost all variables of disease activity. Three patients discontinued therapy because of lack of improvement, 2 patients because of side effects and one patient was lost to followup. Cytofluorographic analysis revealed a significant decrease in expression of a T cell activation antigen (gp 26). Our data suggest that minocycline could be a useful therapeutic agent in RA.

Adult↗
PubMed1992

Followup in psoriatic arthritis: relationship to disease characteristics.

We investigated whether patient disease characteristics can explain differences in patients registered at the psoriatic arthritis clinic before 1987 who were seen regularly (139, "regular") to those who had not been seen for 2 years before 1989 (165, "inactive"). Despite a higher percentage of women in the regular group (p = 0.0058), logistic regression analysis identified no significant differences in disease severity or treatment at the first or last assessment before 1987. A followup study in 1989 revealed that of the 165 inactive patients, 20 had died and 64 had moved away. Of the 81 remaining patients, 18 were not interested, 18 were seeing other physicians, and 45 returned for followup. The latter 45 patients were compared to 85 of the regular patients who were seen during the same period. Both groups demonstrated similar disease attributes at first and last assessments before 1987. At their 1989 assessments, the same degree of disease progression was noted in both groups. Thus, loss to to followup appears to be random, and should not influence further studies of these patients.

Adult↗
PubMed1992

[Composite lymphoma in Sjogren's syndrome with mixed cryoglobulinemia].

A 44-year-old woman with Sjogren's syndrome and mixed cryoglobulinemia developed nodal and pulmonary lymphoma 15 years after the initial diagnosis. The nodal lymphoma was of the composite type and included both monoclonal monocytoid and lymphoplasmacytoid B-cells. The extranodal pulmonary lymphoma included only lymphoplasmacytoid lymphocytes. Complete resolution of the lymphoma was achieved following 3 months of chlorambucil, 5 mg/day, and prednisone, 30 mg/day. Composite lymphoma and monocytoid lymphoma are being encountered more commonly among patients with Sjogren's syndrome. Physicians must therefore become more aware of the existence of both entities.

Adult↗