[Rheumatoid arthritis in the elderly: ten cases report]. / La polyarthrite rhumatoïde du sujet âgé : A propos de dix observations.

BACKGROUND: The occurrence of rheumatoid arthritis (RA) in elderly is frequent. If the reality of a real difference in clinical presentation between younger and older subjects is discussed, the central point remains that the prognosis is not better for the elderly. Finally, conventional treatment is as effective and safe as in younger patients, and the same stringent targets for management of the PR used for young subjects must be applied in the elderly. AIM: To identify the characteristics of RA in the elderly in its epidemiological, clinical, radiological, evolutive and therapeutic. METHODS: We conducted a retrospective study of RA in the elderly aged 65 and over, we've compiled ten cases hospitalized over a period of 4 1/2 years in the service of Internal Medicine, Habib Thameur Hospital (Tunis). RESULTS: There were 8 women and 2 men. The average age was 70.6 years. The onset of arthritis and the disease was progressive in seven cases. An inflammatory syndrome was present in seven cases. Rheumatoid factor was positive in eight cases. Five patients were classified as stage III and IV according to the radiological classification of Steinbrocker. The treatment was based on painkillers and anti-inflammatory drugs in all cases. Long-term treatment was initiated in seven patients. The outcome was favorable in all cases. CONCLUSION: Late-onset RA is a heterogeneous framework in which multiple clinical forms deserve to be individualized and should reflect this diversity, rather than approach to the problem of global RA after 60 years.

[Special features of systemic sclerosis in men]. / Particularités de la sclérodermie systémique chez l'homme.

BACKGROUND: Systemic sclerosis (SS) is a generalized disorder of connective tissue and microvasculature characterized by tissue fibrosis and obliteration of the vessels. Several features of systemic scleroderma in men are discussed in the literature. AIM: To investigate the initial clinical features, evolution and prognosis of systemic sclerosis in men. METHODS: Patients with systemic sclerosis based on ACR's criteria were included. In this retrospective study we compared a cohort of men to a cohort of women, diagnosed between 2000 and 2010 in department of internal medicine. RESULTS: Fifty four patients were included amongst which nine men. The mean follow-up duration was 39.5 months. A higher proportion of cardiac, renal and lung involvement were noted at diagnosis Localized cutaneous sclerosis was predominant in men. CONCLUSION: This work has highlighted several features of systemic sclerosis encountered in men. These results warrant confirmation by analyzing a larger population.

[Takayasu's arteritis in Tunisia: a monocentric study of 11 patients]. / La maladie de Takayasu en Tunisie: etude mono-centrique de 11 cas.

BACKGROUND: Takayasu's disease is a chronic inflammatory arteritis involving large vessels in young women. AIM: To assess the clinical, laboratory, and radiological features and course of Takayasu arteritis in Tunisia. METHODS: This retrospective study analyzed 11 patients with Takayasu arteritis between 1999 and 2010 who met the criteria for inclusion proposed by the American College of Rheumatology (ACR). RESULTS: The file review identified 11 women, with a mean age at diagnosis of 29.1 years (range: 17-50 years). Our series included involvement of the aortic arch and its branches in 8 cases, while only 3 cases of the abdominal aorta. Arterial hypertension due to stenosis of the renal arteries was noted in 4 cases. Aortic insufficiency and pulmonary hypertension were noted in one case. In all, 3 patients had type I disease, 3 type V and one type IV. In all, 4 patients had glucocorticoid treatment, and one needed immunosuppressive therapy. Three patients required surgical intervention. Our patients were followed for a mean period of 67.5 months. Disease remained stable in all patients. CONCLUSION: Despite the small number of our patients, the clinical manifestations, angiographic data and course in our study were similar to those in other reported series.

[Hemophagocytic syndrome: report of 4 cases]. / Le syndrome d'activation macrophagique: a propos de 4 observations.

BACKGROUND: Hemophagocytic syndrome (HPS) is a rare but potentially fatal disease. The diagnosis is based on the combination of clinical and biological signs, requiring histological or cytological research hemophagocytosis and exhaustive etiological investigation. AIM: To report four cases of the HPS in an internal medicine department. CASES REPORT: We report four cases of HPS associated with Still's disease in two cases, with Sjogren syndrome in one case and a severe sepsis in one case. There are three women and one man. The mean age was 34.75 years, with extremes of 21 to 50 years. In all cases, patients were hospitalized for fever of unknown origin. The etiologic research of this fever remained negative. In all cases, patients validated criteria of HPS confirmed by cytological studies. The treatment was based on corticosteroids in all cases and immunosuppressant in one case. The evolution was favorable in two cases and fatal in two cases. CONCLUSION: HPS is a serious, often overlooked, may affect the prognosis and complicating various infectious, neoplastic or autoimmune diseases.

HLA-B, DR and DQ antigens polymorphism in Tunisian patients with ankylosing spondylitis (a case-control study).

The objective of the study is to assess the distribution of HLA-B genes, HLA-B27 subtypes, HLA-DRB1 and HLA-DQB1 alleles in patients with ankylosing spondylitis (AS) and in control subjects in the Tunisian population and to compare their distribution with that found in other countries. This is a case-control study that included 100 consecutive patients (85 males/15 females) with AS according to the modified New York criteria and 100 control individuals. HLA-B, B27 subtypes and class II (DR and DQ) typing of all subjects was performed by polymerase chain reaction amplification with sequence-specific primers (PCR-SSP). HLA-B27 was found in 62% of patients against 3% in controls (P = 0.0000, OR = 52.6, 15.6 < CI < 166.7). On the other hand, B*07 and B*51 were significantly decreased in comparison with controls (P = 0.01, OR = 0.3, 0.1 < CI < 0.8 and P = 0.0000, OR = 0.2, 0.1 < CI < 0.4, respectively). Eight B*27 subtypes were identified in the AS group, but the most frequent ones were B*2702 (32%) and B*2705 (24%). Among HLA-DRB1 alleles, a significant increase in DRB1*11 was found in comparison with controls (P = 0.01, OR = 2.2, 1.2 < CI < 4.5). However, DRB1*13 had a negative association with AS (P = 0.01, OR = 0.4, 0.2 < CI < 0.8). For HLA-DQB1 alleles, a significant positive association with DQB1*03 was observed in AS group (P = 0.03, OR = 1.8, 1.0 < CI < 3.4). Multivariate analysis by logistic regression revealed that DRB1*11 and DQB1*03 had no direct links with the disease, but were dependent on the presence of HLA-B27. Moreover, B*07 and B*51 seemed to have independently a negative correlation with AS, but DRB1*13 seemed to depend on B*51. Haplotypes carrying B27 were significantly associated with AS and those carrying B*07 or B*51 were negatively correlated with the disease. In conclusion, our study confirms that B27 predisposes to AS while B*07 and B*51 are negatively correlated with the disease.

Safety and efficacy of tocilizumab as monotherapy or in combination with methotrexate in Tunisian patients with active rheumatoid arthritis and inadequate response to disease-modifying anti-rheumatic drugs in conditions close to clinical practice.

OBJECTIVES: We aimed to assess the safety and efficacy of tocilizumab as monotherapy or in combination with methotrexate in a routine clinical practice setting in Tunisian patients with RA who did not respond to conventional treatment with disease-modifying anti-rheumatic drugs (DMARDs-IR). METHOD: A total of 51 DMARDs-IR adult patients with moderate to severe RA participated in a phase IIIb, open-label clinical trial. Patients received 8 mg/kg of tocilizumab every 4 weeks in combination with a DMARD or as monotherapy during 24 weeks. The study was extended to 24 other weeks for those who had at least a moderate response at the end of the initial phase. Safety and efficacy of tocilizumab were analysed. RESULTS: Four patients discontinued treatment prematurely due to an adverse event. The most common AEs were hypercholesterolemia (18 cases), increased triglycerides (17 cases), increased transaminases (15 cases), rash (14 cases), neutropenia (7 cases), digestive disorders (3 cases) and respiratory disorders (3 cases). After 52 weeks, 90.5% of patients responded to treatment. At the end of the study, 61.9% of the patients had a mild RA and almost 50% of patients were in remission. Overall, 29.2, 6.3 and 4.3% of patients achieved ACR20, ACR50 and ACR70 responses, respectively. Additionally, the study showed a significant improvement in all individual parameters of ACR core data. CONCLUSIONS: Treatment with tocilizumab was well tolerated and showed a fast and sustained efficacy in Tunisian patients with moderate to severe active RA who had an inadequate response to DMARDs.Key Points• Up to 40% of RA patients remain inadequate responders to a prior csDMARD or a tumour necrosis factor α inhibitor (TNFi) biological agent. A non-TNF biological agent like tocilizumab with or without methotrexate is recommended in those patients.• In this study, tocilizumab treatment improved the number of responders, the number of patients in remission, and the evolution of disease activity. The meaningful clinical improvement seen denotes a rapid and sustained response to treatment.• Tocilizumab presented a favourable safety profile with few withdrawals due to AEs, consistently with what was observed in other trials.• This study provides new information about the safety and efficacy of tocilizumab in a patient population resembling that expected in clinical practice among the Tunisian population.

Magnetic resonance imaging evaluation of the cervical spine in patients with rheumatoid arthritis. Report of 30 cases.

AIM: To investigate by magnetic resonance (MR) imaging the occurence of cervical spine (CS) involvement in rheumatoid arthritis (RA) patients. METHODS: Thirty consecutive unselected patients, who fulfilled the revised American College of rheumatology criteria for RA, were investigated. All patients had a complete physical and laboratory evaluation. Radiological evaluation included hand and wrist x-rays, as well as CS radiographs in anteroposterior, lateral and lateral in full flexion views. In addition, MR (Spin Echo T2-weighted saggital scans, palin and contrast enhanced T1-weighted sagittal and axial scans) was performed in all patients. Hand x-rays were evaluated according to the Sharp score. Disease activity was assessed by disease activity score for 28 joint indices (DAS-28). RESULTS: There were 25 females and 5 males with a mean age of 46.6 years (23-67) and mean disease duration 9 years (1-22). Twenty three patients (76.6%) had positive IgM rheumatoid factor (RF). Five patients presented clinical findings, mainly cervical pain and stiffness of CS (four with positive and one with negative MR), while radiological findings of CS involvement were found in seven patients (23%). Five patients (16.6%) presented MR findings of CS involvement (anterior atlantoaxial subluxation 100%; vertical subluxation 20%; peridental pannus 80%; dens erosion 40%; brainstem compression 20%). Atlantoaxial subluxation correlated with high DAS-28, high level of swellen joint, high level of C-reactive protein and advanced erosive changes of the wrist and hand (high level of Sharp score) in the univariate analysis. CONCLUSION: We conclude that the frequency of CS involvement in RA patients is high. In pateints with active erosive peripheral disease it is very probable to also have some changes in CS. These may be clinically important and in such cases, MR offer valuable information.

[Arterial involvement in Behcet's disease: a series of 7 cases]. / Atteinte artérielle au cours de la maladie de Behcet: a propos de 7 cas.

BACKGROUND: Arterial involvement is rarely described in Behcet disease BD and it is associated with poor prognosis. AIM: We report our experience with a rare and interesting subset of Behcet disease patients with arterial involvement: thrombosis and aneurysm formation types of angio-BD. METHODS: From 1994 to 2008, seven cases of arterial BD with 3 aneurysm formation and 4 thrombosis were found amongst BD patients in the department of internal medicine of Habib Thameur Hospital. RESULTS: Combination of venous and arterial manifestations occurred in 4 patients. All patients were male and the mean age when arterial involvement manifested was diagnosed was about 39.3. The artery most often affected is the aorta followed by the pulmonary arteries. Brain infraction occurred in about 4 cases. Pericarditis and neurological symptoms were more frequently noted in patients with arterial BD than others without vasculo-Behcet disease. All patients underwent corticosteroids and immunosuppressive therapy. Two patients were successfully operated for aneurysms. All patients were alive after a mean of 7.8 years follow-up. CONCLUSION: Arterial involvement is more frequently noted in vasculo-Behcet than venous thrombosis and it's associated with a high morbidity and mortality rate.

[Venous involvement in Behcet's disease: a series of 28 cases]. / Atteinte veineuse au cours de la maladie de Behcet: a propos de 28 cas.

BACKGROUND: Venous involvement is a rare and severe clinical feature in Behcet disease (BD). AIM: We reported our experience with a rare and interesting subset of BD patients with venous involvement. METHODS: From 1994 to 2008, twenty eight cases of venous BD were found amongst BD patients in the department of internal medicine of Habib Thameur Hospital. RESULTS: 45 episodes of venous thrombosis occurred in 28 patients. There were 25 men and 3 women the mean age when venous involvement manifested was diagnosed was about 32.25. Venous thrombosis occur in the first five years following the diagnosis of BD. Pericarditis was more frequently noted in patients with venous BD than others without vasculo-Behcet disease. In contrast digestive and rheumatologic symptoms were more likely observed in patients without vasculo-Behcet disease. Thirteen patients underwent corticosteroids. Relapses occurred more frequently in patients without corticosteroid therapy. CONCLUSION: Venous involvement is more frequently noted in vasculo-Behcet than arterial involvement. Its treatment should include anticoagulation in association with corticosteroids.

[Spinal cord compression due to benign osteoporotic vertebral fracture]. / Compression médullaire par un tassement vertébral ostéoporotique bénin.

BACKGROUND: A neurologic compromise associated with vertebral fractures is generally due to a malignancy causes. Therefore, an osteoporotic vertebral fracture can sometimes cause neurologic complications. AIM: Report a case of neurologic compromise associated with osteoporotic vertebral fractures. OBSERVATION: A-62-year-old man suffered from rheumatoid arthritis since 1985, presented a cervical pain associated with quadriparesia secondary to a C5 osteoporotic vertebral fractures. CONCLUSION: Osteonecrosis may be the cause of neurologic compromise associated with osteoporotic vertebral fractures.

Validation of the Tunisian versions of Bath Ankylosing Spondylitis Functional Index (BASFI) and Disease Activity Index (BASDAI).

BACKGROUND: The Bath Ankylosing Spondylitis Functional Index (BASFI) and the Bath Ankylosing Spondylitis Disease Activity (BASDAI) are the most commonly used instruments to evaluate respectively functioning and disease activity in ankylosing spondylitis (AS). OBJECTIVE: The aim of this study was to translate, adapt and validate these instruments into the Tunisian language. METHODS: The studied population consisted of 68 AS patients (59 males and 9 females). Their mean age was 37.9 years (range: 18-76). The mean disease duration was 13.6 years (range: 1-40). After translation and retranslation the BASFI and BASDAI questionnaires were administrated to the patients and tested for reliability, internal consistency and construct validity. RESULTS: The reproducibility of the indices BASFI and BASFAI was good, the intraclass correlation coefficient for reliability was 0.96 (CCI:0.93-0.97) for the BASFI and 0.93 (CCI:0.90-0.97) for the BASDAI, and the coefficient of internal consistency (Cronbach's alpha) was 0.91 for BASFI and 0.90 for BASDAI. Concerning construct validity, both questionnaires were significantly correlated to each other, to the disease-specific instruments (BASG-s, BASMI, BASRI, ASQoL) and to all domains of the SF-36. CONCLUSION: The Tunisian versions of the BASFI and the BASDAI preserve the metrological properties of the original versions and were easy to use for the assessment of disease status in ankylosing spondylitis.

[Catastrophic antiphospholipid syndrome and rituximab: a new report]. / Syndrome catastrophique et anti-CD20: une nouvelle observation.

BACKGROUND: Catastrophic antiphospholipid syndrome is a distinctly rare dramatic condition characterized by widespread thrombosis of small vessels. Early diagnosis and aggressive therapies are essential in this condition because of its extremely high mortality rate. Therapeutic management include heparine, high dose steroids, cyclophosphamide, plasma exchange, intravenous immunoglobuline, however a number of patients are refractory to treatment. AIM: We review and discuss alternative and emerging treatment options by rituximab for patients who fail or cannot tolerate conventional therapy. CASE-REPORT: A 36-year-old female with a two mounths history of dyspnea, palpitation and chest pain was admitted. Physical examination upon admission revealed a fever, ischemic digital necrosis, scleroderma of the hands and beaking of the nose. Laboratory tests showed normal level of liver enzymes, elevation of creatinine level, lymphopenia, haemolytic anaemia with negative Coombs tests, low platelet count, prolonged partial thromboplastin time. The D-Dimer value was 158 ng/ml. Urinalysis revealed a proteinuria. Antinuclear antibody tests and lupus anticoagulant were strongly positive. Echocardiography revealed severe pulmonary hypertension and pericarditis. There was no pulmonary embolism on thoracic angio tomodensitometry. The diagnosis of catastrophic antiphospholipid antibody syndrome associated with systemic lupus and scleroderma was established. She was treated with anticoagulants, corticotherapy, one pulse of intravenous cyclophosphamide, 2 doses of intravenous immunoglobuline and 5 sessions of plasmapheresis. Because of lack of response 2 doses of 375 mg weekly rituximab i.v. were added but she developed pulmonary embolism, alveolar haemorrhage and she died. CONCLUSION: Effectiveness of Rituximab for the CSAPL should be demonstrated by further studies.

[Leflunomide in the treatment of rheumatoid arthritis]. / Le leflunomide (ARAVA) dans le traitement de la polyarthrite rhumatoide.

The aim of this study was to assess the effect and safety of leflunomide (LEF) in refractory RA and to review the literature on this subject. A one year prospective study was conducted on a group of patients (n:15). Mean duration disease was 6.46 years. Rhumatoid factor was present in 12 cases. Leflunomide was administered at a dos of 20mg/day following a loading dose of 100mg/day for three days. The efficacy of LEF was evaluated on clinical and biological parameters of RA evolutivity at one, 3, 6, 9 and 12 months. Our mean follow up period was about 8 months (2 to 12 months). Good prognostic indicators of disease progression were observed with LEF at one month and later in eleven cases with a good safety. Non serious adverse events were observed with LEF. Our result confirm that LEF may present another therapeutic choice that is efficacious for the long term treatment of refractory RA. Nonetheless, these results must be evaluated on a larger series.