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Alexandra Balbir-Gurman

Publications and source records attributed to Alexandra Balbir-Gurman.

9 recordsLinked to original sources
PubMed2006

Vasculitis in siblings with familial Mediterranean fever: a report of three cases and review of the literature.

Familial Mediterranean Fever (FMF) is characterized by recurrent attacks of self-limited polyserositis and fever. Several types of vasculitis are associated with FMF: polyarteritis nodosa, Henoch-Schonlein purpura (HSP), and protracted febrile myalgia (PFM). We describe three cases of vasculitis in four siblings of a Sephardic Jewish family with FMF and reviewed the literature. One brother and one sister developed severe HSP with intestinal involvement while another brother developed PFM. Genetic tests in three brothers confirmed the M694V mutation on both alleles. Vasculitides may be a clinical feature of FMF with a higher familiar prevalence. MEFV mutations may act as a genetic susceptibility factor for vasculitides in FMF patients.

Adult↗
PubMed2006

Rheumatoid pleural effusion.

OBJECTIVES: To describe the clinical and laboratory features of rheumatoid pleural effusion (RPE) and the diagnostic and therapeutic approaches to this condition. METHODS: The review is based on a MEDLINE (PubMed) search of the English literature from 1964 to 2005, using the keywords "rheumatoid arthritis" (RA), "pulmonary complication", "pleural effusion", and "empyema". RESULTS: Pleural effusion is common in middle-aged men with RA and positive rheumatoid factor (RF). It has features of an exudate and a high RF titer. Underlying lung pathology is common. Generally RPE is small and resolves spontaneously but symptomatic RPE may require thoracocentesis. Rarely, RPE has features of a sterile empyematous exudate with high lipids and lactate dehydrogenase, and very low glucose and pH levels. This type of effusion eventually leads to fibrothorax and lung restriction. Superimposed infective empyema often complicates RPE. Oral, parenteral, and intrapleural corticosteroids, pleurodesis and decortication, have been used for the treatment of sterile RPE. Infected empyema is treated with drainage and antibiotics. CONCLUSIONS: RPE may evolve into a sterile empyematous exudate with the development of fibrothorax. Symptomatic effusions or suspicion of other causes of exudate (infection, malignancy) require thoracocentesis. The "rheumatoid" nature of the pleural exudate in patients without arthritis mandates a pleural biopsy to exclude tuberculosis or malignancy. The optimal therapy of RPE has yet to be established. The role of cytokines in the course of RPE and the possible usefulness of cytokine blockade in the treatment of this RA complication require further evaluation.

Arthritis, Rheumatoid↗
PubMed2006

Anti-cyclic citrullinated protein antibodies as a predictor of response to anti-tumor necrosis factor-alpha therapy in patients with rheumatoid arthritis.

OBJECTIVE: . The treatment of rheumatoid arthritis (RA) has changed dramatically with the introduction of anti-tumor necrosis factor (TNF) agents. Unfortunately, a subset of patients have partial or no response. No measurements were found to predict the efficacy of this therapy. Anti-cyclic citrullinated protein antibodies (anti-CCP) are highly specific and sensitive for RA, and their titer correlates with erosive disease. We investigated the correlation between the efficacy of infliximab therapy and the titer of anti-CCP. METHODS: Thirty consecutive seropositive patients with RA were treated with infusion of 3 mg/kg infliximab on Weeks 0, 2, 6, and 14. Clinical assessment and blood withdrawal were done before each treatment, i.e., at the minimal concentration of the drug. Disease activity was assessed by DAS28 score and by interleukin 6 (IL-6) level. Anti-CCP titer was measured by a commercial ELISA at Week 0 and Week 14. RESULTS: At baseline, 24 patients were positive for anti-CCP antibodies. In most patients there was a significant correlation between clinical response to therapy and anti-CCP titer. The results were especially noteworthy in those patients who showed a sustained and significant decrease in IL-6 levels through the entire period. CONCLUSION: Anti-CCP titer and IL-6 levels might be early predictors of the efficacy of anti-TNF therapy in patients with RA.

Adult↗
PubMed2005

Rheumatoid lung nodulosis and osteopathy associated with leflunomide therapy.

BACKGROUND: Leflunomide (LEF) is indicated in adults for the treatment of active rheumatoid arthritis (RA). LEF inhibits dehydroorotate dehydrogenase, a key enzyme of the pyrimidine synthesis in activated lymphocytes. Among rare adverse effects, fatal interstitial lung disease has been recently reported during treatment of RA with LEF in Japan. Clinical trials outside Japan do not suggest that LEF causes an excess of pulmonary adverse effects. Development and increase of peripheral rheumatoid nodules in typical sites of RA patients following LEF therapy has been recently reported. OBJECTIVES: Two cases with new and accelerated development of rheumatoid lung nodulosis during LEF therapy were described in this study. METHODS: LEF treatment was administered to two male patients (77 and 66 years old) with long-standing active seropositive nodular RA with failure of multiple second line drugs and without lung involvement. Clinical and laboratory assessment using the American College of Rheumatology response criteria, chest computed tomography (CT), quantification of serum rheumatoid factor (RF), and monocyte count of peripheral blood along with routine laboratory follow up were performed on both patients before and during therapy. In case 1, a bone scan was performed due to sustained limbs pain. Open lung biopsy was performed in case 1 and core lung biopsy in case 2. RESULTS: Both patients achieved full clinical remission during 2 months of LEF therapy. In case 1, the first complaints were limbs pain after 10 months of treatment associated with intensive bone uptake on a bone scan consistent with hypertrophic pulmonary osteopathy. Productive cough developed after 3 months of the therapy in case 2. Initially, these complaints were not attributed to therapy. New lung disease was present on CT with cherry-like progressive cavitary nodules, predominantly involving the basal segments of the right lung. The first lung lesions were found by CT 13 months (case 1) and 7 months (case 2) after the beginning of therapy and were erroneously related to bronchiectasia in case 2. In both cases, the lung biopsy showed necrosis surrounded by epithelioid mononuclear inflammation with giant cells, consistent with rheumatoid lung node. The time that elapsed between the beginning of the first symptoms to LEF discontinuation was very long: 13 months in case 1 and 24 months in case 2. Discontinuation of LEF therapy was followed by an arrest in growth of lung nodules, resolution of limb pain, and gradual improvement of bone scan. A significant decrease of monocyte count and RF level in peripheral blood was observed during LEF therapy in both cases. CONCLUSION: For the first time, we described rheumatoid lung nodulosis as complication of successful LEF therapy for RA. Hypertrophic pulmonary osteopathy with severe limbs pain and dry cough were the first manifestations of the lung nodulosis. Monocytopenia during LEF therapy is proposed to be involved in pathogenesis of this rare complication of LEF therapy.

Aged↗
PubMed2004

Endothelin and pulmonary arterial hypertension.

BACKGROUND AND OBJECTIVES: Pulmonary arterial hypertension (PHT) is a potentially fatal disease. The purpose of this article is to review the current knowledge of the role played by endothelin (ET) in PHT and the relevant drug regimens used in the treatment of this condition. METHODS: A detailed search via MEDLINE (PubMed) was performed by using PHT and ET as the key terms. RESULTS: PHT could be a primary or a secondary diagnosis associated with various heart and lung diseases. PHT appears during the late stage of systemic sclerosis and may complicate other systemic diseases such as systemic lupus erythematosus. The vascular endothelium and activation of various mediators and growth factors such as the ET system are thought to play a crucial role in the development of this condition. The pathologic process progresses very rapidly from vasoconstriction to widespread pulmonary vascular obstruction. The use of high doses of calcium channel blockers is of limited value. Life-long anticoagulant therapy is recommended for the treatment of PHT. Currently, the drug being used in PHT therapy is continuous central-venous prostacyclin infusion. Prostacyclin is a strong vasodilator with antiaggregate and antifibrotic properties and has the potential to reduce endothelial injury and to induce vasculature remodeling. This treatment results in improved functional status and increased life span. Unfortunately, its use is accompanied by various side effects, technical difficulties, and high cost. The role of other therapeutic modalities (inhaled prostacyclin, subcutaneous treprostinil, oral beraprost, sildenafil) in vascular remodeling, and the improvement in functional capacity and survival of patients with PHT, are currently under investigation. Bosentan, administered orally, is a recently developed active ET receptor antagonist. It is a promising new therapeutic tool in the treatment of PHT because of its potent vasodilator, antiproliferative, and vascular remodeling activity. CONCLUSIONS: The revolutionary conceptual shift in understanding the pathogenesis of PHT from a vasoconstrictive process to a vasoproliferative one, has led to a modification in the treatment of this disease from the use of vasodilators to the use of drugs with antiproliferative and vascular remodeling activity. Until now, prostacyclin was the only drug of this type available for the treatment of PHT. ET blockade seems to be a reasonable and potential therapeutic option.

Antihypertensive Agents↗
PubMed2003

The handheld dermatoscope as a nail-fold capillaroscopic instrument.

BACKGROUND: The presence of nail-fold capillary abnormalities may be useful in diagnosing several connective tissue disorders, including scleroderma, dermatomyositis, and mixed connective tissue disease, and in differentiating primary Raynaud phenomenon from Raynaud phenomenon due to scleroderma and mixed connective tissue disease. Capillaroscopy, however, usually requires special equipment and may be time consuming. Purpose To investigate the potential use of the unmodified common handheld dermatoscope as a capillaroscopic instrument. Subjects The study included 106 patients who were consecutively referred and a control group of 170 healthy subjects or patients with unrelated skin disorders. METHODS: A nail-fold capillaroscopic examination using a standard handheld dermatoscope was performed on all fingers of each subject. A scleroderma-dermatomyositis pattern was defined as the presence of 2 or more of the following findings in at least 2 nail folds: enlargement of capillary loops, loss of capillaries, disorganization of the normal distribution of capillaries, "budding" ("bushy") capillaries, twisted enlarged capillaries, and capillary hemorrhages (extravasates). RESULTS: A scleroderma-dermatomyositis pattern was found in 19 (70.4%) of 27, 7 (63.6%) of 11, and 4 (50%) of 8 patients with scleroderma, dermatomyositis, and mixed connective tissue disease, respectively. These frequencies were statistically significantly higher than a null percentage of scleroderma-dermatomyositis pattern in the control group (P<.001) and a scleroderma-dermatomyositis pattern in only 1 (4.5%) of 22 patients with systemic lupus erythematosus as well as in 2 (5.3%) of 38 patients with Raynaud phenomenon but without evidence of a connective tissue disorder (P<.01). CONCLUSIONS: The capillaroscopic results obtained with the dermatoscope are comparable to those described with other instruments. Therefore, the unmodified hand-held dermatoscope may be used as a capillaroscopic instrument to detect a scleroderma-dermatomyositis pattern and to help the dermatologist in the clinical diagnosis of connective tissue disorders.

Adolescent↗
PubMed2002

[Anti-TNF-alpha treatment and spondyloarthropathies].

Spondyloarthropathies are characterized by both axial and peripheral joint involvement, by the association with "other diseases" mainly Psoriasis, Crohn's and Anterior Uveitis and by the high prevalence of HLA B-27. While disease modifying drugs, such as Methotrexate or Sulfasalazine, are only partially effective in controlling peripheral arthritis, the treatment of the axial part remained only symptomatic. The recently introduced anti-TNF-alpha drugs Infliximab (Remicade) and Etanercept (Enbrel) for the treatment of Crohn's disease and Rheumatoid Arthritis has been expended to Spondyloarthropathies with highly promising results. The rationale and the early beneficial results of this new approach in spondyloarthropathies are reviewed.

Anti-Inflammatory Agents, Non-Steroidal↗