BackgroundSjögren's syndrome (SS) is a systemic autoinflammatory disease with a well known epidemiological and laboratory characteristics. Its main symptoms are derived from exocrine glands affectation. Like other inflammatory diseases presents an increased incidence of malignancies. The diagnosis of this entity and subsequent identification of factors associated with the occurrence of tumors is important for early detection and managementObjectivesTo analyze the epidemiological, clinical and laboratory characteristics and the occurrence of lymphoma and mortality in a cohort of patients in a tertiary hospital diagnosed with SSMethodsDescriptive analysis of data collected retrospectively of patients with SS (criteria American-European 2002) between November 1989 and November 2012 the Hospital of VigoResultsWe describe 58 patients with SS of which 86% were women with a mean age of 53±15 years.Mean follow-up time was 69 months (range5–343). Mean age at diagnosis was 50 years (range 26–87 years).Only 6 (10%) had a secondary SS associated with Lupus Erythematosus Systemic, Rheumatoid Arthritis, Scleroderma, Mixed Connective Tissue Disease and Sarcoidosis. We do not find any autoimmune disease associated with organ-specific.The 84.5% and 93% had ocular and oral symptoms respectively.The parotid scintigraphy was performed in 25 (43%), which was pathological in 37 (68%).Salivary gland biopsy was performed in 30 (52%), 28 were diagnostic (93%).The most common extraglandular involvement was the joint (50%),followed the skin and lymph nodes (both 12%).24% have Raynaud's phenomenon with capilaroscopia nonspecific. Others, were central and peripheral nervous system (8.6% each one), respiratory (7%) and kidney (3%). None had muscle involvement. Anti-Ro was positive in 83% (59% also had anti-La) and anti-La was positive in only 48%.Antinuclear antibodies and rheumatoid factor were positive in 95% and 67% respectively. 22 (38%) had low C4 and 10 (17%) had low C3. Cryoglobulins were positive in 2 (3%). Only two patients have anti-Ro and anti-La negatives (they had xerostomia and xerophthalmia, parotid scan and biopsy compatible with the diagnosis). The cancer incidence was 10%, 2 were solid tumors (one pancreatic adenocarcinoma and another invasive ductal breast cancer) and 4 were hematologic (diffuse B lymphoma, angioimmunoblastic T lymphoma, Hodgkin's lymphoma and nodal MALT). All those who developed lymphoma they had previous lymphadenopathy. The mortality rate was 12%: 4 neoplasia (2 lymphomas, 1 pancreas and 1 breast), 2 infections, 1 cerebral hemorrhage.ConclusionsThe 89.6% of our series had a primary SS with the same collected clinical and serological features literature. The development of a neoplasm was the leading cause of death (57.1%). In our cohort over half the patients who had lymphadenopathy developed a hematologic malignancy. SS patients have a higher risk for hematologic malignancy, so based on our data, the occurrence of lymphadenopathy it is a warning sign which requires us to actively seek a lymphoproliferativ...

No abstract

No abstract

BackgroundAntineutrophil cytoplasmic antibodies (ANCA) associated vasculitis (AAV) is a necrotizing vasculitis that predominantly affects small vessels and is associated with ANCA specific for myeloperoxidase (MPO-ANCA) or proteinase 3 (PR3-ANCA). The major clinicopathologic variants of AAV include microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic granulomatosis with polyangiitis (EGPA). Recent studies have demonstrated the crucial role of complement activation in the pathogenesis of AAV. However, the clinical characteristics of AAV with hypocomplementemia (HC) still remain unclear.ObjectivesThe aim of our study was to anlyze the demographic, laboratory, treatment and clinical characteristics of AAV using medical records. To compare the AAV patients with and without HC.MethodsRetrospective study of patients with AAV diagnosed and followed in a specific Systemic Autoimmune Diseases and Thrombosis Unit. We defined HC as the state in which at least one of the following was lower than the lower limit of the normal range: Complement 3 (C3), Complement 4 (C4). Demographic, clinical, treatment and evolution data were recorded and analyzed using SPSS 22.0.ResultsThirty-six patients with AAV were included (94,4% MPO-ANCA, 5,6% PR3-ANCA). 8 patients were diagnosed of GPA, 21 of MPA and 3 of EGPA. 61,1% of the patients were males and the mean age at the onset of the disease was 63,11±14,38 years [30–85]. 75% of patients had any vascular risk factors. Renal involvement occurred in 32 patients (88,9%): hematuria in 96,9%, proteinuria in 90,6% (>1 gr 68,7%) and glomerular filtration decreased in 81,25%. Biopsy was performed in 22 patients and a focal and segmental necrotizing glomerulonephritis with extracapillary proliferation was the finding more common. Pulmonary disease (61,1%) included interstitial disease 40,9%, alveolar hemorrhage 22,7% and nodules 18,2%. Other clinical manifestations were constitutional syndrome 36,1% (the main symptom was asthenia); ear, nose, and throat manifestations 33.3%; neurologic involvement 27.8% (the main finding was polyneuropathy); articular manifestations 33.3% and skin lesions 16,7%. All the patients received prednisone in combination with cyclophosphamide (69.4%) or rituximab (13.9%). Azathioprine was used as glucocorticoid-sparing agent (41.7%). 83.3% of the patients needed hospital admission and 6 died (16.7%). Eleven patients (30,6%) had HC at their diagnosis of AAV. Compared to the AAV patients without HC (n=25), we found no significant differences in the outcome or severity of the manifestations (evaluated by Five factor score). The small sample size could in part explain these results.ConclusionsOur patients with HC at diagnosis of AAV did not have different characteristics than those without HC. More studies are needed to determinate if HC is a predictor of poor prognosis in AAV patients.Disclosure of InterestNone declared

No abstract