Behçet's disease with rapidly progressive bilateral optic neuropathy and avascular femoral neck necrosis. Literature review and management update.

Behçet's disease is a multi-systemic vasculitis of small arteriolar and venular vessels, which shows a wide range of clinical manifestations, such as oral and genital aphthosis, erythema nodosum, panuveitis, complex gastrointestinal disorders, the early onset of neurological involvement being a negative prognostic factor in evolution. We present the case of a 36-year-old patient, who was admitted in the Clinic of Rheumatology for recurrent-neglected oral aphthosis, recurrent erythema nodosum, left hip pain, reduction of visual acuity of the right eye, weight loss, profuse sweating, marked fatigability. From the personal history was retained avascular necrosis of right femoral head, with arthroplasty at this level, human leukocyte antigen-B51 (HLA-B51) positive. Ophthalmological evaluation reveals severe bilateral optic neuropathy, with confirmation of neuro-Behçet's disease (NBD) diagnosis, in a Neuro-Ophthalmological Center, based on cerebral nuclear magnetic resonance and cerebrospinal fluid analysis. Associated corticosteroid therapy with Azathioprine was initiated, with no signs of activity and progression of the disease in evolution. The case provides a necessary upgrade of the therapeutic strategies specific to the NBD pattern, emphasizing the importance of the multidisciplinary approach of a patient with complex pathology.

Ultrasonography role in the evaluation of a giant cell tumor of the flexor pollicis longus tendon.

Hand tendons lesions represent a challenge for an accurate diagnosis, an optimal treatment strategy, the description of the lesion and its location being an important step. The non-invasive ultrasound evaluation was demonstrated to be an important diagnostic method in these types of lesions, especially in those situations where clinical evaluation failed to reveal the pathological changes and therefore has an important role in the adequate management.

Ultrasonography in the diagnosis and management of non inflammatory conditions of the hand and wrist.

High resolution ultrasonography has already become an important tool in the diagnosis and management of inflammatory arthritis of the hand and wrist but lately it has been proven to be the method of choice in the evaluation of traumatic lesions of tendons, annular pulleys, nerves, and ligaments and at the same time in detecting foreign bodies. The objective of this paper is to review and describe the current knowledge on these US findings and to highlight the ultrasonography role in the evaluation of non-inflammatory conditions of the hand and wrist.

IL-17 patterns in synovium, serum and synovial fluid from treatment-naïve, early rheumatoid arthritis patients.

BACKGROUND: There are actually becoming controversial data regarding the profiles of interleukin-17 (IL-17) in different pathogenical stages of rheumatoid arthritis (RA). OBJECTIVES: To assessing the IL-17 patterns in synovium, serum and synovial fluid from treatment-naïve early RA patients and to identifying potential correlations with disease activity markers and with synovial histopathological profile. MATERIALS AND METHODS: Serum samples from 30 treatment-naïve early RA patients were evaluated for C-reactive protein (CRP), erythrocytes sedimentation rate (ESR), rheumatoid factor (RF), anti-cyclic citrullinated peptide antibodies (anti-CCP). IL-17A levels were also assessed in serum and synovial fluid (SF). Disease activity score (DAS28) calculation was done for all patients. Control serum and SF samples were obtained from 29 patients with osteoarthritis (OA); control synovium specimens were obtained from eight patients with OA and during surgery for knee tear ligaments. Histopathological (Hp) score, immunohistochemical reactivity for IL-17 were also assessed in synovium of early RA patients and controls. Dependencies between serum and synovial profile of IL-17A and the other parameters were statistically tested. RESULTS: In early RA patients, strong correlations of serum and SF IL-17A levels were found with ESR, CRP, RF, anti-CCP, Hp score and IL-17 synovial immunoreactivity; a good correlation was noted with DAS28 score. Also, strong correlation was noted between serum and SF IL-17A levels. CONCLUSIONS: In early stages of untreated RA, simultaneous IL-17 assessment of serum, SF and synovium might be valuable in defining activity and predictive patterns, given that synovium is highly suggestive for an disease aggressivity and might express specific therapeutically targets.

Power Doppler sonography, a non-invasive method of assessment of the synovial inflammation in patients with early rheumatoid arthritis.

OBJECTIVE: To detect synovitis is important in both the diagnosis and outcome assessment of early rheumatoid arthritis. This study was meant to assess the validity and reproducibility of ultrasonography (US) as a mean of detection for the knee synovitis, by comparing US findings with clinical examination and histopathological findings in synovial membrane. METHODS: The study group included 65 patients with early rheumatoid arthritis - below 12 months from the debut, naive for DMARDs, in whom demographic data - gender, age, disease duration, the number of tender and swollen joints, HAQ score (Health Assessment Questionnaire) and serum samples for CRP, RF, anti-CCP2 antibodies (ELISA, QUANTA LiteTM, CCP IgG, INOVA Diagnostics Inc, USA), VEGF (ELISA, VEGF2, DRG International, IRC, USA) determination were recorded. Disease Activity Score for 28 joints (DAS28) was calculated. PDS signal was scored from 0 to 3 according to the overall expression of PDS findings at the knees. A sample of synovial tissue was obtained in 35 patients during the arthroscopy, and the vascularisation of the synovial tissue was evaluated by immunohistochemistry and was analyzed qualitatively by a pathologist who was unaware of the PDS findings. Written, informed consent was obtained from each patient before entering the study. They all had active synovitis of the knee, ultrasonographically confirmed, with the identification of the target biopsy sites. The study was approved by the Ethics Committee of the University of Medicine and Pharmacy of Craiova. RESULTS: Angiogenesis was evaluated and quantified by immunohistochemical evaluation of synovial VEGF, one of the most specific endothelial growing factors, that proved to correlate significantly with the serum levels of VEGF, DAS28 as well as with the power Doppler sonography (PDS) score. CONCLUSIONS: The statistical analysis of the data showed that PDS could be used as non-invasive marker with predictive value regarding synovial inflammation and disease progression in early forms of the disease as well as a useful method in the assessment of the therapeutic response.

CD20cy and CD45RO immunoexpression in early rheumatoid arthritis synovium.

In this paper, we studied 15 cases of early rheumatoid arthritis presenting with inflammatory lesions in different degrees of evolution. We want to highlight B- and T-lymphocytes in synovial tissue collected from patients diagnosed with early rheumatoid arthritis, to establish the pattern of their distribution, possibly in relation to local neovascularisation to determine the role played by these types of cells. The pathological samples were represented by synovial membrane biopsy fragments, which were examined by histopathological and immunohistochemical methods. We noticed a perivascular distribution of lymphocyte infiltrate, up to formation of lymphoid follicles with germinal centers. There is a close interdependence between B- and T-lymphocytes in these lesions, and their presence in the synovial membrane in relation to newly formed blood vessels facilitates their action and their chemical mediators. Studying the interdependence of different types of lymphocytes and their connection with blood vessels may generate new therapeutic targets.

Osgood-Schlatter disease--ultrasonographic diagnostic.

Osgood-Schlatter disease is a condition that is caused by traction of the muscle-tendon unit at tibial tuberosity, which affects adolescents who exercise. The predisposing factors include rapid growth and physical activity, particularly running and jumping. Osgood- Schlatter disease causes intermittent pain, which can be aggravated by running, cycling and climbing stairs. We present the case of a young boy, soccer player, which presented for pain at the level of the tibial tuberosity. Ultrasonography showed changes at the distal part of the patellar tendon and at the tibial tuberosity, consistent with Osgood-Schlatter disease.

The study of angiogenesis in early rheumatoid arthritis--clinical, immunohistochemical and immunological correlations.

OBJECTIVE: The objective of the study was to analyze several immunohistochemical, histological, and morphometrical aspects of angiogenesis in early rheumatoid arthritis synovium. We aimed to identify possible correlations between the histological and immunohistochemical patterns and the serum levels of VEGF, as well as with clinical and biological markers of disease activity. METHODS: 35 patients with early rheumatoid arthritis below 12 months from the onset, naive for DMARDs, underwent clinical standard examination as well as serum determinations for CRP, RF. anti-CCP2 antibodies and VEGF. DAS28 value has been determined for each patient in order to assess the disease activity. We performed biopsy sampling through arthroscopy, the synovium fragments beeing histopathologically processed, in order to elaborate a total histological score. Immunohistochemical analysis has been performed with quantification of synovial VEGF, VEGF-R1 and CD34 expression. Standard and activated microvascular density (sMVD and aMVD) have been evaluated through double immunostaining (CD34/ VEGF-R1). RESULTS: VEGF and VEGF-R1 have been identified with high prevalence in endothelial cells, in lining and sublining synovial cells, as well as in inflammatory cells. The study focuses on the analysis of aMVD, a valuable parameter, representative for active angiogenesis, which proved to correlate significantly with the serum levels of VEGF, the composite histological score as well as with VEGF-R1 and DAS28. CONCLUSION: The statistic analysis of the data support VEGF-R1 and aMVD as markers with predictive value regarding activity and progression in early stages of rheumatoid arthritis. The validation of preliminary conclusions oblige to continuous research through extending the study group and inclusion of several others biomarkers involved in synovial angiogenesis.

A case of portal cavernoma--associated essential thrombocythemia.

Portal cavernoma is a rare complication of portal vein thrombosis and may be a component of the myeloproliferative hematologic disorders. In the present case report, although the symptoms induced by portal hypertension and portal biliopathy had a concordant evolution with the portal cavernoma, the peripheral thrombocytosis and the medullar expression of essential thrombocythemia became relevant at 18 months.