The relatively high prevalence of systemic lupus erythematosus (SLE) in familial cases supports genetic susceptibility to this disease. Although many advances have been made in the identification of new genes implicated in lupus pathogenesis, to date, there has been no large study of familial SLE. We report what we believe to be the first study of familial SLE in the North African population. The objectives of this study were to determine the main clinical and laboratory features of familial lupus and to compare them to sporadic lupus in a population of Tunisian patients. Fourteen families in which the diagnosis of lupus could be verified in at least two relatives were included in the study. All patients fulfilled four or more criteria defined by the American College of Rheumatology. Twenty-seven patients (23 females and 4 males) with familial SLE among a cohort of 253 SLE patients were found, resulting in a frequency of 10.67%. No significant differences were found between familial SLE cases and their controls in terms of sex ratio, mean age at onset and clinical and serological manifestations, which is consistent with the results of other series reported in the literature. Our results support the importance of carrying out more genetic studies within families of SLE in order to have a better understanding of the genetic and molecular mechanisms of the disease.

Autoimmune Lymphoproliferative syndrome is an inherited disorder manifesting with autoimmune cytopenia, lymphadenopathy and splenomegaly. The differential diagnosis includes infections, autoimmune disorders or malignancies. The disease is characterized by accumulation of double negative (CD3+ CD4-CD8-) T cells (DNT) in the peripheral blood. Here we report the case of 19 years-old girl that was diagnosed as autoimmune Lymphoproliferative syndrome with a literature review.

Pulmonary artery aneurysm must be evoked in front of any hemoptysis in a patient with Behçet disease as it requires urgent immunosuppressive therapy and often surgery.

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Introduction: Pure extra-thoracic sarcoidosis is rare and poorly characterized in the literature. Herein we present features of pure extra-thoracic sarcoidosis, in comparison with sarcoidosis with intra-thoracic involvement, in a population from the south of Tunisia. Methods: Retrospective, descriptive and comparative study of the patients presenting systemic sarcoidosis and attending the Department of Internal Medicine at the Hedi Chaker University Hospital, Sfax, Tunisia, for 21 years, from January1996 to December2016. Results: Twenty-four patients presented pure extra-thoracic sarcoidosis (30% of the cases of systemic sarcoidosis). There was a female predominance (79%). The mean age was 50 years. The main features were polyadenopathies (10 patients:41,7%), cutaneous involvement (10 patients:41,6%), inflammatory polyarthralgia (8 cases:33.3%), general symptoms (6 patients:25%), uveitis (6cases:25%), cholestasis (3cases) and kidney involvement (2cases). Lymphopenia, hypercalcemia, and hypercalciuria were observed each one in 12.5% of the cases, and high angiotensin converting enzyme (ACE) level in 38,5% of the cases. The histological proof was required in all the patients. Statistically significant associations identified were mainly hepatic involvement with general symptoms (p=0.035), peripheral lymphadenopathies (p=0.035) and kidney damage (p=0.022), and cutaneous involvement with articular manifestations (p=0.032). Systemic corticosteroids were used in half of the cases, with a good outcome in 21 cases, and recurrence in two cases. One patient was lost to follow up. The comparison to the form with mediastino-pulmonary involvement showed less lymphadenopathies(p=0.001), less lymphopenia (p=0.006), and less frequent use of corticosteroids(p=0.044). Conclusion: Our series was characterized by the frequency of the pure extra-thoracic form and the diversity of the systemic manifestations.