[Pregnancy in dermatomyositis and polymyositis]. / Grossesse au cours des dermatomyosites et polymyosites.

AIM: To determine the incidence of pregnancies during the inflammatory myopathy (IM), its influence on this disease and the influence of IM on the pregnancy's outcome. METHODS: From 1979 to 2007 we have collected retrospectively 67cases of IM (53 women and 14 men) in the department of internal medicine CHU Hedi Chaker of Sfax (Tunisia). Diagnosis criteria were those and Peter. We have included pregnancies occurring after diagnosis of DM or PM and cases of IM occurring during pregnancy or postpartum. RESULTS: Five women (4DM and PM), average age 30.6 years (range 26 - 41 years) conducted 10 pregnancies (9.4%). The fertility rate is 2 pregnancies per patient. Nine pregnancies occurred after the diagnosis of myositis in 4 women. IM was inactive at conception in all this cas.No flare-up in IM has been noted during these pregnancies. The DM was revealed to the 10th day post-partum in one patient. Therapeutic interruption of pregnancy was needed in 3 cases. 4 pregnancies were completed without incident and resulted in the birth of healthy newborns. Foetal complications were observed in the other two pregnancies occurring in one patient who also presents an APS associated with DM. It was a foetal death in utero and premature delivery of a newborn who died in 3rd day after birth. CONCLUSION: Pregnancy in the MI is rare. Its influence on the activity of the disease is variable, both during the pregnancy than postpartum. The foetal prognosis is good when the the disease is in remission. However foetal complications are important in case of active IM.

Intima-media thickness of carotid artery in patients with Behçet's disease.

BACKGROUND AND AIMS: In this study we investigated the carotid intima-media thickness (cIMT) in Tunisian patients with Behçet's disease (BD), and we evaluated the relationship between traditional and nontraditional cardiovascular risk factors, disease manifestations, disease severity, use of immunosuppressive agents and cIMT in patients with BD. METHODS: Fifty patients with BD were individually matched to 50 control subjects on the basis of age, gender and traditional cardiovascular risk factors. Subjects with diabetes mellitus, evidence of myocardial infarction or cerebrovascular disease were excluded from the study. Patients with arterial involvement were also excluded. We measured serum high-sensitivity C-reactive protein (hsCRP), fibrinogen, creatinine levels and lipid profile. We also measured serum vitamin B12, folate, total plasma homocysteine levels, and HOMA-IR values. We used B-mode ultrasonography to assess the cIMT. RESULTS: cIMT in the BD group was significantly higher than in the control group (0.658 ± 0.112 mm vs. 0.581 ± 0.087 mm, respectively, p <10(-3)). The frequency of plaques in the carotid arteries was similar between study groups. In univariate analyses, the cIMT of BD patients was correlated with age (r = 0.510, p <10(-3)), male gender (p = 0.032), and creatinine clearance (r = -0.421, p = 0.003). It was inversely correlated with HOMA-IR values and serum vitamin B12 level. cIMT values were independent of disease manifestations, disease duration, or corticoid therapy. In the multivariate analysis, only male gender and creatinine clearance remained significantly associated with cIMT. CONCLUSIONS: Our data indicate morphologic evidence of subclinical atherosclerosis in patients with BD. Increased arterial wall thickness was not associated with the disease duration, clinical manifestations and immunosuppressive therapy.

Atherogenic lipid profile in Behçet's disease: evidence of alteration of HDL subclasses.

BACKGROUND AND AIMS: Behçet's 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.

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.

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

Behçet's 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.

Membranous glomerulopathy associated with idiopathic hypereosinophilic syndrome.

Renal involvement is rarely reported and little is known about the renal morphologic changes in the idiopathic hypereosinophilic syndromes. We reported a case of a 52-year-old man with idiopathic hypereosinophilic syndrome associated with membranous glomerulopathy. The eosinophilia and the proteinuria greatly improved with corticosteroid treatment. We review the literature regarding the nephropathy of this hematologic disorder.

Systemic sclerosis in a patient with pityriasis rubra pilaris.

Pityriasis rubra pilaris (PRP) is a rare, chronic erythematous squamous disorder of unknown etiology. It has been found in association with several autoimmune diseases, including thyroiditis, myositis, myasthenia gravis and vitiligo. Herein we report a case of systemic sclerosis in a patient with classic adult pityriasis rubra pilaris. A 38 year old woman with classic adult type 1 pityriasis rubra pilaris (PRP) developed progressive skin thickening of the trunk, face, upper and lower extremities after 2 years of PRP treatment with topical emollients and steroids. Clinical examination and immunological findings were consistent with SSc. Co-existence of these two rare conditions is documented for the first time.

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.

[Takayasu arteritis in southern Tunisia: a study of 29 patients]. / Maladie de Takayasu au sud tunisien: étude de 29 cas.

OBJECTIVES: This study aimed to assess the clinical, laboratory, and radiological features and course of Takayasu arteritis in Tunisia. METHODS: This retrospective study analyzed 29 patients with Takayasu arteritis between 1996 and 2006 who met the criteria for inclusion proposed by the American College of Rheumatology (ACR). RESULTS: The file review identified 25 women and 4 men, with a mean age at diagnosis of 35.4 years (range: 18-65 years). Our series included 93% with involvement of the aortic arch and its branches, while only 24% involved renal arteries and 21% the abdominal aorta. We had no case with cardiac or pulmonary involvement. In all, 67.7% had type I disease, 10.7% type IIb, 3.6% type IV and 25% type V. Hypertension was recorded in 38%. No tuberculosis was observed. In all, 22 patients (75.8%) had glucocorticoid treatment, and 5 (17.2%) needed immunosuppressive therapy. Two patients with renal artery stenosis had endoluminal angioplasty and four patients (13.7%) required surgical intervention. Our patients were followed for a mean period of 80 months. Disease remained stable in 18 patients (64.2%). CONCLUSION: The clinical manifestations, angiographic data and course of our patients were similar to those in other reported series. We found no relation between Takayasu arteritis and tuberculosis.

[Adult Schönlein-Henoch purpura associated with epidermoid carcinoma of the lung]. / Association purpura rhumatoïde de l'adulte et carcinome épidermoïde du poumon.

INTRODUCTION: Association between Schönlein-Henoch purpura and neoplasm can suggest the responsibility of tumour antigens in the genesis of the vasculitis. We report a new case of squamous cell carcinoma associated with Schönlein-Henoch purpura and we discuss the reality of this association. CASE REPORT: We report the case of a 50-year-old man who presents Schönlein-Henoch purpura with a purpura of lower limbs, joint involvement, gastrointestinal lesions and IgA renal mesangial deposits. The patient received three intravenous methylprednisolone pulses followed with oral corticosteroids. Six months later, while the vasculitis was in remission, the patient presented a squamous cell lung carcinoma. He was treated by chemotherapy and local radiotherapy. At the late follow-up, the neoplasm was incompletely resolved. CONCLUSION: The neoplasm could be responsible of the development of the Schönlein-Henoch purpura. The discovery of this systemic vasculitis in an elderly patient should warrant a deep screening for an occult neoplasm.

Wegener's granulomatosis presenting as multiple bilateral renal masses: case report and literature review.

Wegener's granulomatosis (WG) is a disease of unknown etiology characterized by necrotizing granulomatous vascularitis. The upper and lower respiratory tract and kidney involvements are very common; however, its presentation as bilateral renal masses is unusual. We report a case of a 59-year-old female patient who presented with multiple bilateral renal masses. The patient presented with sinusal and ocular symptoms suggestive of WG, and positive antineutrophil cytoplasmic antibodies (c-ANCA) with an anti-PR3 pattern. Histopathologic examination of the renal biopsy specimen revealed granulomatous inflammation with vasculitis and fibrinoid necrosis. The patient management, including prednisone and cyclophosphamid, induced a marked improvement of the renal masses. This case illustrates that WG should be considered in the differential diagnosis of renal masses.

Clinical and immunological manifestations of systemic lupus erythematosus: study on 146 south Tunisian patients.

The objective of this study was to determine the main clinical and laboratory features as well as the morbidity and mortality of systemic lupus erythematosus (SLE) in a population of patients predominantly from the south of Tunisia. A retrospective review of a well documented population of 146 patients with SLE was undertaken. All patients fulfilled four or more criteria defined by the American College of Rheumatology. The mean age at presentation was 29.2 years (range 6-55) and the mean duration of follow-up was 62 months (range 0.25-374). Musculoskeletal (84.2%) and mucocutaneous (75.3%) were the most frequent clinical manifestations. Antinuclear antibodies were detected in 97.3%, anti-DNA antibodies in 69.2% and anti-Sm in 39.2% of the patients. Anti-cardiolipin antibodies and lupus anticoagulant were ob-served respectively in 71.6% and 37.8% of the patients. The five-year survival rate in our series was 92%. Renal involvement and thrombocytopenia were associated with poor prognosis (p< 0.05). The clinical and immunological characteristics of our SLE patients are largely comparable to most major studies. Main differences included prominent major organ damage and high pre-valence of anti-Sm and anti-cardiolipin antibodies.

[Mortality and prognostic factors in 146 patients with systemic lupus erythematosus in southern Tunisia]. / Mortalité et facteurs de mauvais pronostic au cours du lupus érythémateux systémique dans une série de 146 cas du Sud tunisien.

OBJECTIVES: The objective of this study was to analyze the survival rate, causes of death, and prognostic factors in patients with systemic lupus erythematosus (SLE), mainly from southern Tunisia. METHODS: This retrospective study examined the records of 146 Tunisian patients with SLE, all meeting at least 4 of the American College of Rheumatology criteria for this diagnosis. RESULTS: The mean age at presentation of these patients (126 women and 20 men) was 29.2 years (range: 6-55), and the mean duration of follow up 62 months (range: 3 days-374 months). Eleven patients (7.5%) died during follow-up. The 5- and 10-year survival rates in this cohort were both 92.1%. The most frequent causes of death were infections and disease activity, each accounting for 4 deaths. Logistic regression showed that nephrotic syndrome was an independent prognostic factor for death (p=0.032, OR=5.64). CONCLUSIONS: Our results confirmed the improvement in recent years of survival rates among SLE patients. Infections and disease activity remain the most common causes of death. Nephrotic syndrome is independent risk factor for mortality.