[Clinical, radiographic and biologic particularities of ankylosing spondylitis in Tunisian patients according to the presence or the absence of the HLA B27 and its sub-types]. / Particularités cliniques, radiologiques et biologiques de la spondylarthrite ankylosante en Tunisie selon la présence ou l'absence du HLA B27 et de ses sous-types.

To assess the clinical, radiographic and biologic particularities of ankylosing spondylitis (AS) in Tunisian patients according to HLA B27 and its sub-types statute. This was a case-control study that included 100 patients (85 males/15 females) with AS according to the modified New York criteria. Demographic, clinical, AS specific indexes, radiographic and biologic parameters were determined. HLA-B and B27 subtypes typing of all subjects were performed by PCR-SSP. Patients mean age was 38.4 years +/- 12.6 HLA-B27 was found in 62% of patients. The comparison of B27 positive and B27 negative patients revealed a correlation of B27 with age, male gender, family history of spondylarthropathies, age at disease onset, acute onset of the disease, inaugural spinal involvement, uveitis, bilateral and destructive hip arthritis as well as a high score of mSASSS. The most frequent sub-types of HLA B27 were B*2702 (49.2%) and B*2705 (36.3%). No significant difference of the clinical presentation of the disease or severity factors was found among these patients. This study confirmed the contribution of the HLA B27 to the determination of the clinical presentation of AS. The variability of factors linked to B27 may be explained by the polygenic model of the disease.

[Scapulohumeral hydatidosis: a new case in Tunisia]. / L'hydatidose scapulohumérale: à propos d'une nouvelle observation en Tunisie.

Osseous hydatidosis is reported in only 0.5-2.5% of the cases. The scapulohumeral localization is extremely rare. A 39-year-old woman, born in a rural area of Tunisia, presented swelling and tenderness of the left shoulder with limited motion 7 days after a minor trauma. Plain radiographs, CT and MRI showed osteolytic scapulohumeral lesions, cortical rupture and multiple cysts in the muscles, which were suggestive of hydatidosis. Indirect haemagglutination test using hydatid antigen was positive (1/280). The patient refused radical surgery and underwent resection of axillary cysts. Albendazole was given in the recommended dose but was stopped immediately due to hepatic toxicity. Scapulohumeral hydatidosis is extremely rare, often invasive and behaves like a locally malignant bone tumour. Its treatment is also difficult.

[Cervicobrachial neuralgia revealing neurosarcoma]. / Névralgie cervicobrachiale révélatrice d'un neurofibrosarcome (MPNST) cervical.

Malignant peripheral nerve sheath tumor (MPNST) or neurofibrosarcoma, previously described as malignant Schwannoma or neurosarcoma, is an extremely rare cause of malignancy localized in the neck. Half of reported cases occurred in patients with neurofibromatosis in Von Recklinghausen disease type I. Typical features include high grade malignancy and a tendency to recurrence and distant metastases. We report the case of a 56-year-old woman with neurosarcoma of the neck, which was revealed by a cervicobrachial neuralgia. The physical examination found a mass on the left side of the neck. Plain radiographs showed osteoarthritis. MRI showed a well-defined paravertebral mass. Pathologic diagnosis was neurosarcoma. Radiotherapy was delivered.

[Langerhans' cell histiocytosis of the dorsal spine. A rare etiology of spinal cord compression]. / Localisation dorsale d'une histiocytose langerhansienne responsable d'une compression médullaire.

INTRODUCTION: Langerhans cell histiocytosis of the thoracic spine is a rare condition in adults. It is a serious condition with an increased risk of spinal cord compression. EXEGESIS: A 55-year-old man complained of lumbar and back pain. He developed a spastic paraparesis. Magnetic resonance imaging of the spine showed a spinal cord compression due to bone and epidural tumoral lesions of the eleventh and twelfth thoracic vertebrae, located in the posterior elements of the spine. A surgical biopsy showed an infiltrate of eosinophilic cells, positive for CD1a in immunohistochemistry studies. Final diagnosis was therefore langerhans cell histiocytosis of the thoracic spine complicated by spinal cord compression. The patient received glucocorticoid therapy combined with vinblastine followed by local radiotherapy and etoposide. However, the neurological deficit persisted and the patient finally died. CONCLUSION: Langerhans cell histiocytosis is a rare cause of spinal cord compression.

Tuberculosis of the greater trochanter: a report of three cases.

Trochanteric tuberculosis represents less than 2% of all musculo-skeletal tuberculosis. The diagnosis is difficult especially if abscess and fistula are missing. The authors report 3 cases of trochanteric tuberculosis. The diagnosis was established, respectively, 4, 9 months and 1 year after the beginning of the symptoms. The tuberculosis was plurifocal in all cases. Diagnosis was based on the presence of caseum granuloma in the first case, positive Lowenstein culture in the second case and on clinical and paraclinical arguments in the third one. Healing was obtained after medical treatment alone. The authors discuss the potential role of the newer imaging modalities in diagnosis of trochanteric tuberculosis and the indications of medical and surgical treatment.

[Vertebral hydatidosis: medical imaging and management. A case report]. / L'hydatidose vertébrale: apport de l'imagerie moderne et actualités thérapeutiques. A propos d'un cas.

Vertebral hydatidosis (VH) is uncommon. It is the most frequent skeletal manifestation of echinococcosis. It is also the most serious one. We report a case of VH revealed by a kyphoscoliosis with dorsal pain and point out the contibution of new medical imagings in the diagnosis of VH and in the patient follow-up. We stress on the difficulties of the care management of this disease and its poor prognosis because of the risk of neurological damages and frequent recurrences.

[Disseminated form of Rosai-Dorfman disease. A case report]. / Forme multifocale d'une maladie de Rosai-Dorfman. A propos d'une observation.

INTRODUCTION: Extranodal involvement in sinus histiocytosis with massive lymphadenpathy (SHML) or Rosai-Dorfman disease is common, seen in 43% of cases. We present a case of disseminated form of SHML with thyroid, renal, bone and lung involvement. EXEGESIS: A 53-year-old woman presented with cervical lymphadenopathy and a large palpable mass in the submandibular area. A thyroid nodule was palpable. Laboratory data showed an increase of the erythrosedimentation rate and a polyclonal hypergammaglobulinemia. Histopathological examination of a cervical lymph node biopsy showed typical features of SHML. CT scan showed enlargement mediastinal lymph node, mass of the right lung and infiltrative mass in the right renal hilium. Radiographs revealed lytic lesions in the right proximal tibia and left patella. Histopathology of a biopsy from the kidney mass and thyroidectomy displayed typical characteristics of SMHL. The patient was treated by steroids with a markedly regression of the kidney and renal masses. CONCLUSION: Multiple extranodal involvement in SMHL is rare, the prognosis may be poor when lesions are massive and involve vital organs.

[Destructive polyarthritis in Behcet's disease. Apropos of a case and review of the literature]. / Polyarthrite destructice au cours de la maladie de Behçet. A propos d'un cas et revue de la littérature.

The authors report a case of a 37 years old man with Behçet's disease since 7 years who develops a destructive chronic polyarthritis involving wrists, hands and one knee. The parallel evolution between recurrent orogenital ulceration and arthritis added to the absence of other associated destructive rheumatism permit to link this polyarthritis to Behçet disease. In spite of the few reports, the occurrence of destructive arthritis can't exclude absolutely the diagnosis of this affection.

[Aggressive angiomyxoma of the perineum and pelvis. A report of two cases with pelvic and para-testicular localization]. / Angiomyxome agressif périnéo-pelvien. A propos de deux observations de localisation pelvienne et paratesticulaire.

Two cases of aggressive angiomyxoma of the pelvis and perineum are described; the first occurred in 32 year old woman who had a vulvar mass, the second in a 78 year old man who presented inguinal hernia. This recently described entity is characterized by its microscopic appearance (low cellularity, loose myxoid stroma and prominent vascular component), and propensity to local aggressivity, and common recurrence after surgery.

[Primary hypertrophic osteoarthropathy without cutaneous involvement (Currarino's disease)]. / Ostéoarthropathie hypertrophiante primitive sans atteinte cutanée (maladie de Currarino).

INTRODUCTION: Hypertrophic osteoarthropathy (HOA) is a syndrome associating hippocratic fingers, arthropathy and periostosis of long bones. Currarino's disease, considered at present as a clinical form of primary HOA, is characterized by the absence of pachydermia. OBSERVATION: A 24-year-old Caucasian man, consulted for a painful swelling of both ankles that had developed over the past year. Clinical examination revealed hippocratic fingers without pachydermia. The ankles were swollen. The X-rays showed periosteal apposition and an acro-osteolysis. In view of this triad: arthropathy, hippocratic fingers and periostosis, primary HOA without cutaneous involvement or Currarino's disease was diagnosed. The search for a secondary cause remained negative. Clinical improvement was obtained after 15 months with non-steroidal anti-inflammatory drugs (NSAID) and colchicine. COMMENTS: Although exceptional, primary HOA without cutaneous involvement is a genetic disease which must not be ignored.

[Rosai-Dorfman disease with orbital and rhinopharyngeal localizations. A case report]. / Maladie de Rosai-Dorfman. A propos d'une localisation orbitaire et rhinopharyngée.

OBJECTIVES: We present a case report of Rosai-Dorfman disease with multiple extranodal involvement and favorable outcome after steroid therapy. MATERIAL AND METHODS: Rosai-Dorfman disease was diagnosed in a 17-year-old man with orbital, lacrymal, and rhinopharyngeal localizations. Histopathologic examination of lymphadenopathy biopsy revealed the diagnostic of Rosai-Dorfman disease. We present the histological findings leading to diagnosis and radiological data. RESULTS: Conservative treatment with oral steroids resulted in the resolution of cervical lymphadenopathy and extranodal lesions. The patient was free of problems nine mouths after discontinuation of his treatment, demonstrating its efficacy and safety. CONCLUSION: Rosai-Dorfman disease is a benign disorder and steroid treatment must be initiated in cases with significant cosmetic deformity.

[Erosive spondylarthropathy in hemodialysis. A case report]. / La spondylarthropathie erosive des hemodialyses. A propos d'un cas.

We report a case of 67-year-old woman with 11-year history of hemodialysis that complain of neck pain associated with cervico-brachial neuralgia. Imaging finding simulate infectious spondylitis. We consider the diagnosis of destructive spondylarthropathy in hemodialyzed patient since etiologic investigation was negative and RMN finding was suggestive. Our patient was partially improved with symptomatic treatment.

[Rheumatic spondylodiscitis in spondylarthropathies]. / Spondylodiscite rhumatismale au cours des spondylarthropathies.

The involvement of enthesis like disc space is the typical lesion of spondylarthropathies. Out of 240 patients with spondylarthropathies, 10 (7 M, 3 W; mean age: 37 years) have spondylodiscitis, affecting a total of 13 disc spaces: 1 cervical, 2 thoracic, 10 lumbar. The mean duration of spondylarthropathy at the time of diagnosis of spondylodiscitis is 10 years. Spondylodiscitis has a variable clinical presentation: painful (6 cases), asymptomatic (4 cases). Its coexistence with typical radiographic signs of spondylarthropathy added to the exclusion of bacterial origin, permit to link it to spondylarthropathy. Its evolution is generally favourable.

[Systemic manifestations of chronic hepatitis C]. / Les manifestations systemiques des hépatites virales C chroniques.

It's well known that hepatitis C virus (HCV) related chronic liver disease may be associated with various extra hepatic disorders. These manifestations can revealed the hepatic disease. We review the available data on the conditions and asses their clinical implications: vascular, cutaneous, articular, neurological or renal disorders. There is no correlation between these extra hepatic manifestations and the severity of liver disease. Several recent studies have established a strong link between HCV infection and essential mixed cryoglobulinemia but some other extra hepatic associations are just fortuitous. Others datas are necessary to better analyze these extra hepatic disorders and to offer the beneficial treatment of patients with chronic hepatitis C.

[Synovitis caused by plant thorn and chronic polyarthritis. Apropos of a case and review of the literature]. / Synovite à piquant végétal et polyarthrite chronique. A propos d'un cas et revue de la littérature.

A 54 year old male developed plant thorn synovitis of the knee followed five months later by nonerosive seronegative polyarthritis. The occurrence of chronic inflammatory joint disease shortly after plant thorn synovitis is exceedingly rare. The relationship between the two events remains to be clarified.

[Research on Parvovirus B19 infections and chronic articular manifestations in a Tunisian hospital]. / Recherche du Parvovirus B19 dans les manifestations articulaires chroniques en milieu hospitalier tunisien.

Parvovirus B19 infection is often associated with acute and chronic joint diseases thus suggesting an etiologic role for the virus in these pathologies. In this work, we looked for a possible correlation between Parvovirus B19 infection and certain types of chronic inflammatory rheumatisms. We therefore, screened a population of 100 patients with different chronic inflammatory rheumatismal affections for serological markers of Parvovirus B19 infection. All patients were Tunisians of both sexes, who presented at the service of Rheumatology of the Charles Nicolle Hospital, Tunis. One hundred blood donors were taken as controls. Specific Immunoenzyme Assays of the ELISA type (Biotrin International, France) were used to detect anti-Parvovirus IgG and IgM. On the other hand, viral DNA was sought by nested PCR in synovial fluid from 14 patients. The data obtained indicate that specific anti-Parvovirus B19 IgG was detectable in the sera of 80.7% of patients and 43% of controls. In contrast, none of the sera was found positive for specific IgM antibodies. Synovial fluid samples could be collected from 14 anti-Parvovirus B19 seropositive patients and were tested for the presence of viral DNA. None of the samples was found positive. The results of our serological study reinforce the hypothesis that Parvovirus B19 infection is associated with rheumatismal joint affections. However, the lack of detectable viral DNA in synovial fluid of the tested seropositive patients points to an indirect role of the virus in these joint disorders.

Comparative study of indices of activity evaluation in rheumatoid arthritis.

INTRODUCTION: Choosing between the different indices of activity evaluation in RA is often difficult considering the very heterogeneous clinical expression of the disease. The objective of our study was to evaluate the level of similarity between SDAI, CDAI, DAS28-(ESR) and DAS28-(CRP) indices in the evaluation of RA activity. PATIENTS AND METHODS: In this transversal study, a total of 100 patients with RA responding to the ACR 87 criteria were followed up for a period of 20 months. The correlations between the four indices were studied through the Pearson's correlation coefficient (r). The similarity between these tools was evaluated through Kendall's (K) "tau" similarity coefficient. RESULTS: The 87 female and 13 male patients (sex ratio: 6.7F/1M) were of a mean age of 52.9±11.6 years (17-77) and have been living with RA for a mean of 8.3±9 years (2 months-41 years). The DAS28-(ESR) mean score was 5.53±1.46 [1.25-8.05]. The DAS28-(CRP) mean score was 5.01±1.44 [1.68-7.81]. The CDAI mean score was 30.72±15.04 [2-62] and that of SDAI was 28.86±15.86 [2.3-71.3]. A positive, statistically significant correlation was noted between the four indices of RA activity. The level of similarity between the different indices was good (K variation between 0.758 and 0.943). DAS28-(ESR) allowed classifying the patients in the same category of disease activity than DAS28-(CRP) in a proportion of 85%. This proportion was 88% when comparing DAS28-(ESR) to CDAI and SDAI, respectively. Regarding DAS28-(CRP) and CDAI, these two indices classified the patients in the same category in a proportion of 80%, compared to 87% regarding DAS28-(CRP) and SDAI. Finally, CDAI and SDAI classified the patients in the same category in a proportion of 92% with an excellent level of similarity. CONCLUSION: Different evaluation indices of RA activity are currently available. DAS28 is the most used. CDAI and especially SDAI have a good level of similarity with DAS28. Their advantage is to be simple and quick, and seem therefore well adapted to the follow-up of outpatients.