Background Primary Sjögren's syndrome (pSS) is a systemic disorder characterized by lymphocytic infiltration and progressivedestruction of exocrine glands. The inflammatory process extends beyond the exocrine glands and can potentially affect any organ.pSS patients may develop a large number of systemic manifestations including neurological, renal, pulmonary and hematologicalmanifestations. While sicca features primarily affect the quality of life and cause local complications in the mucosa involved,systemic involvement marks the disease prognosis.
Objectives The goal of this work is to study the demographic, clinical, serologic features and outcomes of Tunisian patients withpSS.
Methods It is a single-center retrospective study including all the cases of pSS diagnosed since 1998 until 2011 in InternalMedicine Department from Tunis in Tunisia. All the patients fulfilled the 2002 American-European Consensus Group (AECG)criteria for pSS.
Results The study include 31 patients: 4 men and 27 women. The age average was 51 years. Dryness of the mouth and eyes wasobserved in all the cases. Immunological assays showed that the anti-SSA and anti-SSB antibodies were both positive in 96% ofpatients. Extra-glandular manifestations were found in 87% of cases, revealing the disease in 77% of patients. In 45% of pSS cases,the Sjögren's syndrome was revealed only by systemic manifestations without any glandular symptom.Musculoskeletal manifestations, were the most frequent, observed in 67% of the cases. Leucocytoclastic vasculitis and neurologicalinvolvement was noted in 23% of patients each one. Raynaud's phenomenon was observed in 13% of cases as was the pulmonaryinvolvement. Interstitial nephritis was diagnosed in 3 patients. Hematological manifestations were marked by autoimmune hemolyticanemia in 3 cases and thrombotic thrombocytopenic purpura (TTP) in one case.Corticosteroids were given in 10 of our patients. Non-steroidal anti-inflammatory drugs (NSAID) were used for 12 patients andantimalarial for 3 cases.The disease outcomes was marked by good evolution. But, malignant lymphoma occurs in a woman after 40 months of primary Sjögren's syndrome past history.
Conclusions During the pSS, the most common clinical manifestation is mouth and eyes dryness. But glandular manifestations arenot always in the foreground. pSS has no one specific clinical or biological sign that makes it diagnosis easy. Clinicians should notignore this condition. Because it displays the highest incidence of malignant lymphoproliferative disorders among autoimmunediseases, pSS should be diagnosed as early as possible.
Disclosure of Interest None declared
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