Makram Frigui

Clinical and diagnostic value of ribosomal P autoantibodies in systemic lupus erythematosus

OBJECTIVE To analyse prospectively the diagnostic sensitivity and specificity as well as the clinical relevance of ribosomal P (anti-P) autoantibodies in a large cohort of SLE patients. METHODS The anti-P autoantibodies were evaluated in the serum of 200 Tunisian SLE patients at disease onset and 130 various control subjects by a sensitive immunodot assay. A complete laboratory evaluation and clinical examination were performed in each SLE patient. During the follow-up, the patients were regularly monitored for clinical parameters. Global SLE activity was measured by the ECLAM. RESULTS The sensitivity and specificity of anti-P testing for SLE were 23.5 and 98.4%, respectively. The anti-P-positive samples 14/47 (29.8%), 27/47 (57.4%) and 5/47 (10.6%) were negative for anti-dsDNA, anti-Sm or both antibodies, respectively. The anti-P-positive patients showed more active disease activity and a much higher prevalence of arthritis. An association between IgG aCLs and anti-P antibodies was also found. However, anti-P antibodies were not associated with neuropsychiatric manifestations or lupus nephritis. CONCLUSION This study does not seem to confirm the described association of anti-P antibodies with neuropsychiatric manifestations of SLE. However, it supports the anti-P antibody association with arthritis and disease activity as well as the presence of aCL. Based on our study and other related studies, we propose that, akin to anti-Sm and anti-dsDNA, anti-P antibodies detected by one agreed method may be considered for inclusion as a criterion for the classification of SLE.

Destructive arthritis in Behçet’s disease: a report of eight cases and literature review

Behçet’s disease (BD) is a multisystemic disease with typically non‐erosive and non‐deforming joint manifestations. The occurrence of destructive arthritis in Behçet’s disease has rarely been reported. Here we attempt to define the epidemiological, clinical and radiological features of this unusual type of osteoarticular manifestation of BD. We retrospectively reviewed the medical records of 553 patients with Behçet’s disease seen over 25‐year period in our department of Internal Medicine (Sfax‐Tunisia). All the patients fulfilled The International Study Group of Behçet’s Disease criteria. Patients with destructive arthritis (defined by radiological changes: erosions and/or geodes and/or global narrowing of the joint space and/or ankylosis) were included in this study. Rheumatologic manifestations were observed in 71.1% patients. Eight patients (1.4% overall, 2% among patients with rheumatologic manifestations) had presented with destructive arthritis. The joint symptoms involved the knee in two cases, the wrist in one case, the elbow (one case), the sternoclavicular joint in two cases, the foot in one case and the tarsal scaphoïd in one case. There was recurrent arthritis at the same joint in the majority of cases. X‐ray examinations revealed radiological changes: global narrowing of the joint in one case (knee), narrowing of the joint with geodes in three cases (knee, sternoclavicular), isolated geodes in two cases (tarsal scaphoid, foot) and severe lesions with ankylosis in two cases (two elbows, right wrist). Joint manifestations are common in patients with BD, but destructive arthritis is rare.

Acute pancreatitis as initial manifestation of adult Henoch-Schönlein purpura: report of a case and review of literature

Abdominal pain observed in Henoch-Schönlein purpura (HSP) is usually attributed to edema and hemorrhage in the small bowel wall, secondary to a small-vessel vasculitis. Pancreatitis secondary to HSP is extremely rare. Here we report a 53-year-old man presented with acute pancreatitis that developed into characteristic rashes seen during HSP at the second day of the clinical onset, together with arthritis and glomerulonephritis. HSP is a rare and benign cause of acute pancreatitis. This complication can occur as an initial manifestation of HSP. Elevated serum amylase level can be considered as the early diagnostic tool for HSP pancreatitis. The patients with HSP who have abdominal pain as their chief complaint should be evaluated for pancreatitis, by routine serum amylase and abdominal computed tomography scan, to plan the specific treatment and avoid unnecessary surgery.

Atherogenic Lipid Profile in Behçet’s Disease: Evidence of Alteration of HDL Subclasses

BACKGROUND AND AIMS Behçets disease (BD) is an inflammatory vasculitis, most common in the Mediterranean area and Asia. Evidence for accelerated atherosclerosis in BD has been observed. The relationship between cardiovascular risk factors and accelerated atherosclerosis in patients with BD is still controversial. The aim of this study was to evaluate the lipid profile and to investigate the low-density lipoprotein (LDL) size and the distribution of high-density lipoprotein (HDL) subpopulations in BD patients. METHODS Thirty six BD patients were compared to 36 healthy controls. Total cholesterol (TC), triglycerides (TG) and HDL-cholesterol (HDL-C) levels were measured using standard techniques. HDL subclasses and LDL-C size were estimated using polyacrylamide linear gradient gel electrophoresis. The LDL-C/HDL-C ratio was also calculated. High-sensitive C-reactive protein (hsCRP) level was measured by a turbidimetric method. Homocysteine (Hcy) level was determined using a liquid chromatography tandem mass spectrometry (LC/MS/MS). RESULTS In BD patients, HDL-C levels as well as its subfraction levels were decreased (respectively, p <10(-6) and p <10(-3)). Percentage of HDL2 subpopulation was also decreased (p=0.02). HDL3 subfraction was significantly higher (p=0.02). The LDL-C/HDL-C ratio and CRP level were increased (respectively, p=10(-4) and p=0.003). TC was correlated with CRP. HDL-C and its subfractions were correlated with CRP and TG levels. HDL subparticle percentages were also correlated with age. CONCLUSIONS Our findings of a reduction of HDL-C and HDL2 subpopulation and an increase HDL3 subclass and a higher LDL-C/HDL-C ratio may be considered as important predictors of cardiovascular events in BD patients.

Deep vein thrombosis associated with acute brucellosis: a case report and review of the literature

To the Editor, Human brucellosis remains the most common zoonotic disease worldwide, with more than 500,000 new cases annually. It is still a serious and uncontrolled public health problem in many countries, including Tunisia. Human brucellosis is a multisystem disease that may present with a broad spectrum of clinical manifestations and its complications can affect almost all organs and systems, with varying incidence. Vascular complications of Brucella infection have rarely been reported in the medical literature. By searching MEDLINE, eight cases of deep vein thrombosis (DVT) associated with acute brucellosis have been reported [1-8]. We describe a new case of DVT in the left leg in association with acute brucellosis. A 52-year-old Tunisian man was admitted to our hospital with a 4-day history of fever and left calf pain associated with myalgia and night sweats. He also reported the ingestion of raw sheep milk. The patient was febrile, to 38.3℃. Homan sign was positive on the left. The circumference of his left calf was 3 cm larger than that of the right. Palpation of the right sacroiliac joint was painful. The rest of the physical examination was unremarkable. A Doppler ultrasound study showed a thrombosis in the left femoral vein. Laboratory examinations revealed the following results: white blood cell count 7,300 cells/mm3 (neutrophils, 49%), platelet count 275,000/mm3, erythrocyte sedimentation rate 30 mm, and C-reactive protein 43 mg/dL. Results of other routine biochemical blood tests were all normal. Protein C, protein S, antithrombin III, and activated protein C resistance activity were within normal ranges, and anticardiolipin and lupus anticoagulant were negative. The chest X-ray and abdominal ultrasound were normal. An anteroposterior X-ray of the pelvis showed grade II sacroiliitis of the right sacroiliac joint. Standard tube agglutination testing of initial samples were positive for antibodies to Brucella (titer, 1/640). Based on the history of ingestion raw sheep milk, fever associated with myalgia and night sweats, the presence of sacroiliitis, and a positive Wright test, a diagnosis of acute Brucella infection associated with a DVT in the left leg was made. The patient was treated with rifampicin 900 mg/day and doxycycline 200 mg/day combined with low molecular weight heparin. The treatment durations for both anticoagulant and brucellosis were extended to 3 months. After a follow-up period of 9 months, the patient was asymptomatic and, during this period, no recurrence of brucellosis or DVT was observed. Vascular complications of brucellosis are rare; the arteries are more affected than veins. Arterial complications of brucellosis include aneurysm formation in diverse arteries, such as the aorta, brachial, tibioperoneal, and cerebral arteries, with or without underlying endocarditis. Cutaneous vasculitis has also been noted. Vein thrombosis is a rare vascular complication in acute brucellosis. In 1973, Romem et al. [8] reported the first case of brucellosis complicated with central vein thrombosis. Since this publication, seven further cases of vein thrombosis due to brucellosis have been reported. Table 1 shows demographic data and clinical and laboratory findings of the eight case reports of brucellosis complicated with vein thrombosis. Thrombosis involved the portal vein [7], the central retinal vein [8], the cerebral vein [6], and the lower limb veins [4] in one case each. Acute brucellosis was associated with DVT in the legs in four patients (three men, one woman). The diagnosis of DVT in the leg was made before the onset of other clinical features of brucellosis in one case [2] and concomitant with the diagnosis of brucellosis in two [3,5]. The duration between DVT diagnosis and the occurrence of brucellosis clinical features was 4 weeks. One patient had a history of brucellosis [1]. Table 1 Summary of all reported cases of deep vein thrombosis associated with brucellosis Suggested mechanisms by which brucellosis may cause DVT include induction of inflammation by the infectious process in adjacent tissues, direct endothelial damage, granulomatous endophlebitis, compression from a local soft tissue mass or abscess, induction of a transient hypercoagulable state, and an immune reaction in the vessel wall to a Brucella antigen. In the present case, protein C, protein S, and antithrombin III levels, and activated protein C resistance activity were normal, and no antiphospholipid antibodies were detected. Also, no local infection adjacent to his left leg deep veins was observed in his illness. Thus, it is possible that granulomatous endophlebitis or a possible immune reaction in the vessel wall to a Brucella antigen was responsible for the patients DVT. DVT is a rare complication of brucellosis. We report a new case of leg thrombophlebitis revealing acute brucellosis. The present case and the others reported previously suggest that brucellosis must be considered in patients suffering with DVT, particularly in patients from Brucella-endemic areas.

Tumeur myofibroblastique inflammatoire récurrente avec triple localisation, rénale, rétropéritonéale et ganglionnaire

Inflammatory myofibroblastic tumors are uncommon and benign tumors with unknown aetiology. First reported in the lungs, the inflammatory myofibroblastic tumors have been observed in other locations, especially in the abdomen and the pelvis. We report a 14-year-old adolescent female, who presented sequentially an inflammatory pseudotumor of lymph node, the left kidney and the retroperitoneum. Extrapulmonary inflammatory myofibroblastic tumors are mesenchymal solid tumors. They are frequently circumscribed and confined to a single organ. The recurrence of some inflammatory myofibroblastic tumors and their expression of chromosomal abnormalities found in some types of lymphoma suggest that some of these lesions constitute a true neoplastic process.

Telangiectasic mastocytosis with systemic sclerosis.

Cutaneous mastocytosis is characterized by increased number of mast-cells in skin that release mediators causing pruritus, urticaria, and flushing [1]. Most cases of mastocytosis involve the skin, whereas systemic mastocytosis is less frequent. Systemic mastocytosis has been reported in association with other diseases such as haematological malignancies and solid tumours [2]. Systemic sclerosis (SSc) is a connective tissue disorder characterized by excessive collagen deposition in the dermis and internal organs and by vascular hyperreactivity and obliteration phenomena [3]. Although interleukin 4 (IL-4) level is of major importance in the pathophysiologic features of both mastocytosis and SSc, we know of only one case of an association of cutaneous mastocytosis and SSc [4]. Here we report on two patients with SSc who showed skin lesions associated with mast-cell infiltrates compatible with a diagnosis of cutaneous mastocytosis.

Coexistence of Behçet's disease with ankylosing spondylitis and familial Mediterranean fever: a rare occurrence

Behçets disease (BD) and familial Mediterranean fever (FMF), which are two separate diseases sharing some clinical features, may also coexist in the same patient. Further investigations are needed to understand whether this coexistence is due to either chance or geographical distribution patterns of these diseases or to common etiopathogenetic characteristics. Spondylarthritis as part of the clinical picture in these two diseases has been questioned and probably it is not a prominent characteristic of any of them. We report a 35-year-old Tunisian man who had an association of BD, FMF and Human Leukocyte Antigen (HLA) B27 positive ankylosing spondylitis. Although that spondylarthritis is an infrequent joint involvement of FMF and BD, it must be looked for in case of association of these diseases.

Cas cliniqueTumeur myofibroblastique inflammatoire récurrente avec triple localisation, rénale, rétropéritonéale et ganglionnaireRecurrent inflammatory myofibroblastic tumor with renal, retroperitoneal and lymph node involvement

Inflammatory myofibroblastic tumors are uncommon and benign tumors with unknown aetiology. First reported in the lungs, the inflammatory myofibroblastic tumors have been observed in other locations, especially in the abdomen and the pelvis. We report a 14-year-old adolescent female, who presented sequentially an inflammatory pseudotumor of lymph node, the left kidney and the retroperitoneum. Extrapulmonary inflammatory myofibroblastic tumors are mesenchymal solid tumors. They are frequently circumscribed and confined to a single organ. The recurrence of some inflammatory myofibroblastic tumors and their expression of chromosomal abnormalities found in some types of lymphoma suggest that some of these lesions constitute a true neoplastic process.