Poster Session : PS 0388 ; Infectious Disease ; Infectious Spondylodiscitis: The Importance of Clinical Suspicion

( Marta Perez De Lis Novo ),( Alexandre Perez Gonzalez ),( Melania Estevez Gil ),( Emma Filgueira Davila ),( Julian Fernandez Martin ),( Roberto Perez Alvarez ) 대한내과학회 2014 대한내과학회 추계학술대회 Vol.2014 No.1

Background: To analyse the epidemiological, clinical, diagnostic and therapeutic features related to the development of spondylodiscitis in a cohort of Spanish patients. Methods: A retrospective analysis of 75 cases of infectious spondylodiscitis diagnosed in university hospital between 2000 and 2013. Results: We studied 75 patients with a mean age of 63 + / - 20 years, of which 67% were male. Time average evolution of disease prior to consultation was 4.2 months. The main symptom was back pain or back pain (90%) The most frequent location was the lumbar spondylodiscitis (74%), 60% of patients had one or more associated diseases (Diabetes:20%; COPD: 18%; tumors: 17%). Furthermore, 50% of them suffered from heart disease, being the most frequently hypertensive. 23% of patients carried immunosuppressive therapy, mainly corticosteroids (90%). In 53 patients (70%) could be performed a certain diagnosis, with Staphylococcus aureus the most frequent (41%) followed by S. epidermidis (17%), and Streptococcus (15%). Blood cultures were positive in 60% of cases and surgical biopsy was performed in 37% of the patients. 8 cases had endocarditis criteria, 6 of which were fulfi lled by S. aureus. MRI was performed in 75% cases, being in all cases, pathological. The average length of treatment was 12 weeks, with the most commonly used antibiotics cloxacillin, vancomycin and ceftriaxone. 18% of patients underwent surgery, performed laminectomy and drainage of abscesses. During evolution 6 patients died. 10 patients (8%) had sequelae neurological. Conclusions: Spondylodiscitis is a rare disease in adults, but with high morbidity and mortality due to the diffi culty of diagnosis. Insidious development and the lack of specifi city of the symptoms it interferes with the diagnosis and treatment early, so a high index of suspicion is essential.

Slide Session :OS-NEP-05 ; Nephrology : Ormond`s Disease (Retroperitoneal Fibrosis) and Idiopathic Mesenteritis in Internal Medicine: Possible IgG4-Related Diseases?

( Marta Perez De Lis Novo ),( Roberto Perez Alvarez ),( Soledad Retamozo ),( Pilar Brito Zeron ),( Xavier Bosch ),( Antoni Siso Almirall ),( Jaume Villalta ),( Ricardo Alvarez Vijande ),( Alfonso Lope 대한내과학회 2014 대한내과학회 추계학술대회 Vol.2014 No.1

Background: To analyse the possible existence of features associated with IgG4-related disease (IgG4-RD) in patients diagnosed with retroperitoneal fi brosis or mesenteritis. Methods: Study cohort including patients diagnosed with retroperitoneal fi brosis and/ or mesenteritis by imaging studies (CT) in a university hospital during the last 20 years. Results: A total of 22 patients diagnosed with retroperitoneal fi brosis (15 patients) or mesenteritis (7 patients) are described (6 women and 16 men, with a mean age at diagnosis of 64.1 years, range: 46-82. The main clinical manifestations present at diagnosis were abdominal/back pain (n=14), general malaise/fever (n=7) and the presence of edema/thrombosis (n = 6); in one patient, diagnosis was made incidentally by imaging techniques. CT showed infl ammatory masses affecting retroperitoneal (n=17), mesenteric (n=7) and vascular structures, including iliac arteries (n=3), aorta (n=2), renal arteries (n=2) and cava (n=2). In 7 (32%) patients, involvement of other organs suggestive of IgG4-RD (liver/bile tract in 2 cases, periaortitis in 2 cases, pleura, lymph nodes and skin in 1 each, respectively) was found. Diagnosis was confi rmed by biopsy in 8 patients (36%); in any patient, studies to rule out IgG4-RD were performed. The main treatments included corticosteroids (n=17), immunosuppressive agents (azathioprine in 5, methotrexate in 1 and mycophenolate in 1), surgery (n = 5) and the placement of urinary catheters (n = 4). After a mean follow-up of 73 months, 7 patients died (32%) patients (4 of them due to septic shock). Conclusions: Ormond`s disease is a rare clinical entity but associated with a signifi cant morbidity and mortality. His recent inclusion in the clinical spectrum of IgG4-RD diffi cult their retrospective diagnosis, although this study and others recently published suggest that about half of the cases could correspond to IgG4-RD.

Poster Session : PS 0640 ; Respiratory Medicine ; Infl uence of Epidemiological Profi le in the Clinical Expression of Sarcoidosis: Study of 160 Patients with Systemic Disease

( Pilar Brito Zeron ),( Marta Perez De Lis Novo ),( Roberto Perez Alvarez ),( Jacobo Sellares ),( Soledad Retamozo ),( Xavier Bosch ),( Fernanda Hernandez ),( Josep Manel Santos ),( Antoni Siso Almira 대한내과학회 2014 대한내과학회 추계학술대회 Vol.2014 No.1

Background: To analyse the infi uence of the epidemiological profi le in the disease expression of a large series of patients with sarcoidosis. Methods: Study cohort including 160 patients diagnosed with sarcoidosis in the last 10 years. The diagnosis of sarcoidosis was based on clinical criteria and imaging studies, together with the histopathological demonstration of non-necrotizing granulomas, and excluding other granulomatous diseases, mainly infectious (tuberculosis). Results: There were 106 women and 54 men, with a mean age at diagnosis of 46.87 years. The main clinical manifestations present at diagnosis were respiratory symptoms (40%), followed by general symptoms (31%), skin lesions (29%) and the incidental fi nding of imaging fi ndings in asymptomatic patients (18%). 37% of patients had adisease onset <40 years. This early onset of disease was reported mainly in males (44% vs 28%, p=0.027) and was associated with a higher frequency of general symptoms (42% vs 25%, p=0.023), erythema nodosum (29% vs 16%, p=0.041), uveitis (14% vs. 5%, p=0.05) and joint involvement (17% vs 5%, p=0.014), but with a lower frequency of interstitial lung disease (25% vs 42%, p=0.028) and splenic involvement (0% vs 7%, p=0.037). With respect to the differential presentation of the disease according to gender, women diagnosed with sarcoidosis had a higher mean age at diagnosis (48.8 vs 42.8 years, p=0.017), a higher frequency of skin involvement (35% vs 13%, p=0.003) and a lower frequency of pulmonary (34% vs 52%, p=0.022), hepatosplenic (2% vs 9%, p=0.044) and nervous system (3% vs 13%, p=0.032) involvements in comparison with males. Conclusions: An early disease onset (<40 years) was mainly associated with general and cutaneous involvement, while sarcoidosis in men affects more frequently vital organs.

Slide Session : OS-HEM-03 ; Hematology : Hemophagocytic Syndrome in Internal Medicine: Iden-tification of Infectious Triggers, Therapeutic Manage-ment and Mortality in 88 Patients (REGHEM-GEAS-SEMI)

( Pilar BRITO ZERÓN ),( Marta PEREZ DE LIS NOVO ),( Roberto PÉREZ ALVAREZ ),( Pedro MORAL MORAL ),( Aleida MARTÍNEZ ZAPICO ),( Guadalupe FRAILE ),( Eva FONSECA ),( María VAQUERO HERRERO ),( Angela RUI 대한내과학회 2014 대한내과학회 추계학술대회 Vol.2014 No.1

Background: To analyze the therapeutic management and the main epidemiological and clinical characteristics related to survival in a large series of patients with hemophagocytic syndrome (HS) diagnosed in Departments of Internal Medicine. Methods: In June 2013, the Study Group of Autoimmune Diseases (GEAS-SEMI) creates a national registry of patients with HS. Patients were diagnosed according to the fulfillment of the criteria of the Histiocytosis Society in 1991 and updated in 2004. Results: At June 15, 2014, the REGHEM registry included 88 patients with HS, 35 (40%) men and 55 (60%) women, with a mean age at diagnosis of 49.16 years (range 12- 84 years). During the admission that led to the diagnosis of SH, acute infections were identified in 50 (57%) patients, including viruses (n=20), bacteria (n=13), mycobacteria (n=8) and parasites/fungi (n=13). Patients were treated with corticosteroids (n=40), etoposide (n=12), cyclosporin A (n=12), methotrexate (n=4), tacrolimus (n=2) rituximab (n=3), intravenous immunoglobulins (n=2) and chemotherapy (n=5). A total of 44 (50%) patients died. The main factors associated with mortality were analytical parameters at diagnosis such as the presence of severe anemia (Hb <9 g/L, 89% vs 66%, p=0.01), platelet count <100,000/mm3 (96% vs 64%, p<0.001), neutropenia <1000/mm3 (59% vs 39%, p=0.044), and bacterial infections as precipitating agent (77% vs 45%, p=0.034); a clinical presentation including lymphadenopathy was associated with a lower mortality (59% vs 39%, p=0.044). Conclusions: Hemophagocytic syndrome is a severe multisystemic disease associated in nearly 50% of cases with an acute infection, and usually requires vital support in intensive care units. Despite this and the use of a complex therapeutic approach, half of the patients died. The main prognostic factor identified is the presence at diagnosis of severe cytopenias and the coexistence of bacterial infections.

Slide Session : OS-RHEU-08 ; Rheumatology : Minimally-Invasive Biopsy of Minor Salivary Glands in Internal Medicine: Searching for Systemic Infi ltrative Diseases

( Pilar Brito Zeron ),( Marta Perez De Lis Novo ),( Soledad Retamozo ),( Lluisa Alos ),( Albert Bove ),( Hoda Gheitasi ),( Belchin Kostov ),( Antoni Siso Almirall ),( Roberto Perez Alvarez ),( Manuel 대한내과학회 2014 대한내과학회 추계학술대회 Vol.2014 No.1

Background: To analyze the safety and utility of minor lip biopsy by using the minimally invasive technique, performed in the Department of internal medicine, in patients presenting with immunonegative sicca syndrome. Methods: Prospective analysis of 122 patients (100 women, mean age 60 years) with sicca syndrome and negative anti-Ro/La antibodies in which minimally invasive biopsy of minor salivary glands was performed. Results: The most common histopathological feature consisted of lymphoplasmacytic infi ltration in 57 (47%) patients, followed by fi brosis in 46 (38%) and acinar atrophy in 32 (26%); the type of lymphoplasmacytic infi ltrate was detailed in 53 patients (21 lymphocytic, 20 plasmacytic, and 12 lymphoplasmacytic). According to the lymphocytic classifi cation of Chisholm and Mason, 53 (44%) were classifi ed as normal, 42 (35%) grade I, 11 (9%) grade II, 8 (7%) grade III and 7 (6 %) grade IV. Histopathologic diagnosis were: normal in 46 patients, non-specifi c sialadenitis in 42, primary Sjogren`s syndrome in 26 (15 as the fi nal diagnosis, 11 and probable), and other diagnoses in 6 (fat infi ltration in 5, systemic amyloidosis in 1); no cases of infi ltration by granulomas or IgG4+ were found. The presence of fi brosis was detected more frequently in women (43% vs 14%, p=0.014); the existence of fi brosis (67.70 vs 55.76 years) and acinar atrophy (69.50 vs 56.71 years) correlated with increasing age. No correlation between the degree of infi ltration and diagnostic or immunological features was found. Only 8% patients reported transient adverse events. Conclusions: In 21% of patients with an immunonegative sicca syndrome, a diagnosis of primary SS was confi rmed by minimally invasive biopsy, whereas 35% of patients disclosed a non-specifi c chronic sialadenitis, which could refl ect a “mild” SS that have no place in the current classifi cation criteria of this disease.