Acute myocardial infarction and late diagnosis of antiphospholipid syndrome

Acute myocardial infarction and late diagnosis of antiphospholipid syndrome

Abstracts surge of SBP was in 31 patients (52 %), morning surge of SBP between 34 and 55 mm Hg was in 24 patients (40 %) and morning surge above 55 m...

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Abstracts

surge of SBP was in 31 patients (52 %), morning surge of SBP between 34 and 55 mm Hg was in 24 patients (40 %) and morning surge above 55 mm Hg was in 5 patients (8 %). Conclusion: Nearly half of the patients with clinically stable RA and treated or newly diagnosed untreated hypertension have increased morning surge of SBP. Morning surge of SBP is potentially involved in increased risk for developing progressive cardiovascular disease in patients with RA.

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doi:10.1016/j.ejim.2013.08.297

ID: 127 Hematologic events in the primary Sjögren's syndrome: Cohort of 155 patients H. Boudjelida, A. Berrah Internal Medecine, CHU BAB EL OUED, Algiers, Algeria

doi:10.1016/j.ejim.2013.08.295

ID: 102 Phenomenon of cartilage micro-crystallization in hip osteoarthritis and avascular necrosis of the femoral head M. Kabalyka, A. Dubikova, T. Petrikeevaa, A. Korabtsovb, I. Kuzminc a

Department of Internal Disease, Pacific State Medical University, Vladivostok, Russian Federation b Department X-ray Methods of Research, Far East Geological Institute, Vladivostok, Russian Federation c Department of Traumatology, Regional Clinical Hospital, Vladivostok, Russian Federation

Objective: To examine the phenomenon of articular cartilage micro crystallization in patients with hip osteoarthritis (OA) and avascular necrosis (AVN) by the method of X-ray diffraction analysis. Methods: Eighty patients with OA and AVN (48 females and 32 males) undergoing total hip replacement between December 2010 and December 2012 were included in the study. 20 controls that underwent arthroplasty performed after femoral neck fractures were included in the group (8 females and 12 males). Mineral cartilage characterization was achieved by means of critical-point drying and carbon evaporation of the specimens to fix the surface, followed by scanning electron microscopy equipped with energy-dispersive X-ray. Results: Such phenomenon as articular cartilage mineralization was identified in 92% of patients with end-staged OA and in all patients with AVN. Analysis of articular cartilage in patients with OA grade III revealed hydroxylapatite crystals (HA) in 65, 8% and calcium pyrophosphate in only 8%. The frequency of occurrence of crystals in patients with OA grade III was 76.3%. In OA patients graded as IV, the frequency of occurrence was 94.4% and the fact is that HA crystals were more often found in this group than in a group graded as III — 77%. In a group of patients with AVN, crystals were detected in 100% of studies and most found were HA crystals — 83.4%. Interestingly, controls showed articular cartilage with HA crystals detected in a quarter of patients, whereas other crystals or their aggregates were not found. Mean age of the patients was 56.23 ± 1.09. Mean HHS score was 52.76 ± 1.98 and VAS score was 8.7 ± 0.75. And there was no statistically significant difference between patients with OA and AVN. At the same time a direct correlation between HHS score in patients with OA and AVN and frequency of mineralization was detected (r = 0.672; p b 0.05). X-ray crystallographic analysis proved a predominant meaning of basic calcium phosphate crystals not pyrophosphate calcium which is known as an etiological factor in microcrystalline arthritis. Conclusions: We state the fact that such phenomenon as articular cartilage mineralization was identified in 92% of patients with end-staged OA and in all patients with AVN. X-Page in 8 of 20 ray crystallographic analysis proved a predominant meaning of BCP crystals not pyrophosphate calcium which is known as an etiological factor in microcrystalline arthritis. In the light of new data, the existing pathogenesis concepts of OA and AVN need revision and that will influence the diagnostic and treatment strategies for these diseases in future.

Introduction: The Sjögren Syndrome (SS) is a systemic disease characterized by lymphoplasmacytic infiltration of the exocrine glands responsible for dry syndrome. This infiltration may spread to other organs and provide systemic manifestations which can be severe. The frequency of hematological manifestations in SS is poorly specified. The aim of our work is to determine the frequency of this event in the SS. Patients and methods: 155 cases of SS primitive collected prospectively over a period of five years (2003–2008). All patients meet the revised europeen criteria (2002). 155 patients (140 women and 15 men: Sex ratio: 9/1); average age = 48.8; delay diagnosis = 76 months, range (3–420 months); the duration of the evolution of SS = 34 months (3–180 months); the average follow-up time = 42 months, range (1–72 months). Sicca syndrome remains isolated in 16% of the cases. The hematologic disease was defined by the presence of abnormal blood counts and/or achieving a hematopoietic organ (lymphadenopathy, splenomegaly). Results: 19 patients (12%) developed lymphadenopathy. Essentially superficial cervical seat (17/19) diffuses (cervical lymphadenopathy, axillary and inguinal) in 2 cases. In 3 cases the superficial lymph nodes are associated with médiatisnales lymphadenopathy and in 2 cases with abdominal lymphadenopathy. Splenomegaly: Presents at diagnosis in 6 patients, and during follow-up in 11 patients. The evolution of the original SS was complicated by non-Hodgkin lymphoma in 6 patients (4%). Cytopenias: 17 patients (11%) with leukopenia, and lymphopenia was noted in 6% of the cases (n = 10). In 5 cases (3%), neutropenia is found, and a case of severe neutropenia to report. Thrombocytopenia is present in 15 patients (10%). Inflammatory anemia was noted in 40 patients (26%), and hemolytic anemia positive coombs is observed in less than 1% of cases (n = 1). Monoclonal gammopathy is found in 5 patients (3%). She appeared secondarily in two patients. Conclusion: Hematological manifestations in SS are common, it is necessary to locate and monitor because they can be predictive of malignant transformation.

doi:10.1016/j.ejim.2013.08.298

ID: 132 Acute myocardial infarction and late diagnosis of antiphospholipid syndrome V. Gomesa, C. Capelaa,b, C. Rodriguesc, F. Gonçalvesc a Autoimmune Disease Unit, Department of Internal Medicine, Hospital of Braga, Braga, Portugal b Life and Health Sciences Research Institute, School of Health Sciences, University of Minho, Braga, Portugal c Department of Internal Medicine, Hospital of Braga, Braga, Portugal

Objective: Antiphospholipid syndrome (ASP) is defined by the occurrence of arterial or venous thrombotic events, often multiple, and recurrent fetal losses, frequently accompanied by a moderate thrombocytopenia, in the presence of antiphospholipid antibodies. The prevalence is around 40–50 cases per 100,000 persons. Due to this

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Abstracts

high prevalence and relatively intuitive diagnosis owing to the typical form presentation in most cases, one would expect its early suspicion by health professionals from all fields of medicine and surgery. The current presentation has the purpose to alert for early recognition of this syndrome because of the associated therapeutic implications and the potential risk of fatality when the diagnosis is delayed. Methods: Revision of one clinical case and literature review were performed. Results: A 44 year-old Caucasian woman with sudden precordial chest pain has been diagnosed with acute myocardial infarction with ST segment elevation of the inferior wall. Percutaneous coronary intervention revealed a large occlusive thrombus in the middle segment of the right coronary artery. Extensive distal embolization occurred during thrombus aspiration. The patient has a history of 3 episodes of deep venous thrombosis (at age 34, unilateral, and at age 36, bilaterally and simultaneously), being under oral anticoagulation with warfarin (INR target of 2.0–3.0) since the latter event. At age 41, she was suspected to have an acute arterial ischemia in the 4th and 5th finger of the left hand. At age 42, under oral anticoagulation levels, she suffered left ulnar artery thrombosis. She has a history of 2 late fetal losses. The only living son was 33 weeks premature at birth. She is a smoker and also suffers from Graves disease and morbid obesity. The previous thrombotic events had been explained to her as secondary to her obesity and peripheral venous insufficiency. She has complaints of longstanding arthralgias in small and large joints with morning stiffness of about 1 h and oral sicca symptoms. Two days before the current episode, the anticoagulation was suspended due to iron deficiency anaemia secondary to metrorrhagia. The study of autoimmunity and other thrombophilia revealed: anti-beta2 glycoprotein-antibody IgM 57 UA and IgG 103 UA, anticardiolipin antibody IgG 30 U/mL, lupus anticoagulant ratio 1.60, antinuclear antibodies 1/80 cytoplasmic pattern, rheumatoid factor 26 UI/mL, sedimentation rate 55 mm/h, homozygosis for the C677T mutation of the MTHFR gene, and no thrombocytopenia. Conclusions: The APS is a potentially devastating disease. Any combination of vascular occlusive events may occur in the same individual and the time interval between them also varies considerably. Deep vein thrombosis and miscarriages are the most common manifestations of APS. Myocardial infarction, digital gangrene and arterial thrombosis in arms are much rarer manifestations (5.5%, 3.3% and 2.7%, respectively). The approach of severe and recurrent thrombotic events despite oral anticoagulation is a complex issue. The authors wish to highlight this case for his academic importance and discuss the diagnosis of APS (primary APS or associated with other diseases, such as systemic lupus erythematosus or autoimmune thyroid disease), the management of comorbidities and future therapeutic implications.

the peripheral nervous system, kidney, skin and gastrointestinal tract, but PAN can virtually affect any organ. Involvement of the central nervous system is rare (5 to 10%). In certain circumstances, its presentation is extremely aggressive. The current presentation has the purpose to point up a case of idiopathic PAN with aggressive central nervous system (CNS) disease and discuss the therapeutic and prognostic implications. Methods: Revision of one clinical case and literature review were performed. Results: Male Caucasian, 41 years old, previously healthy, who developed in a few months, myalgias in the lower limbs, muscle weakness, fever and significant weight loss. One month after the onset of the symptoms, a sudden right motor deficit showed up. The magnetic resonance angiography revealed acute left lenticular cerebral infarction and signs of previous stroke in the right caudate nucleus. Two months after, he developed livedo reticularis and hypertension. His serum panel showed, by this time, increased inflammatory parameters, elevated transaminases, unchanged renal function, no positivity for immunological study (including antineutrophil cytoplasmic antibodies) and negativity for serologies (including hepatitis B virus). On the presumption of vasculitis, he underwent cycles of methylprednisolone and began oral corticosteroid therapy, with significant improvement. Muscle biopsy was performed and showed “vascular formations with multiple small vessels with diameter of capillaries, showing healing aspects of vasculitis.” Four months after the onset of symptoms and after tapering the corticosteroid dose, acute bilateral testicular pain and hypoesthesia of the left foot arise. The electroneuromyography study was compatible with mixed sensorimotor peripheral mononeuropathy. Over the next months, involvement of additional nerves (hypoesthesia of the right foot and 1st to 3rd fingers of the left hand), compatible with multiple mononeuropathy, was observed. The patient met the criteria for PAN. Oral cyclophosphamide 100 mg was added to steroid therapy because of severe and glucocorticoid-resistant disease. However, initially, with advancing neurological symptoms (hypoesthesia of the 4th and 5th fingers of the right hand and lateral side of the right leg) and worsening of the general state with nonspecific constitutional manifestations, despite of negative inflammatory parameters. He complicated also with deep venous thrombosis and spinal lipomatosis with no neurological compression, obesity and osteopenia, secondary to chronic steroid therapy. One year after the presentation of the disease and the beginning of glucocorticoid therapy, the patient maintains the need for 15 mg/day of prednisolone and cyclophosphamide 100 mg/day (since 9 months), with disease control maintained for 4 months. Conclusions: This clinical case represents an aggressive neurological form of PAN with stroke and worsening mononeuropathy multiplex. CNS involvement is a poor prognostic sign and the expected mortality rate at 5 years is 26%. Future therapeutic and prognostic implications are discussed.

doi:10.1016/j.ejim.2013.08.299

doi:10.1016/j.ejim.2013.08.300

ID: 134 Polyarteritis nodosa with central and peripheral neurological involvement V. Gomesa, C. Capelaa,b, C. Rodriguesc, F. Gonçalvesc

ID: 150 Behcet's disease and neurological impairment: Common or uncommon presentation? N. Lorenzoa, S. Diaz Nicolasb, B. Alonso Ortiza, S. Moreno Casasa, L. Salas Reinosoa, Y. Miranda Bacallaob, Z. Cordoba Sosaa, S. Suarez Ortegaa

a Autoimmune Disease Unit, Department of Internal Medicine, Hospital of Braga, Braga, Portugal b Life and Health Sciences Research Institute, School of Health Sciences, University of Minho, Braga, Portugal c Department of Internal Medicine, Hospital of Braga, Braga, Portugal

Objective: Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis involving small- or medium-sized arteries. It is a rare disease, with an annual incidence that ranges from 0 to 1.6 cases per million inhabitants in European countries. Commonly there is an involvement of

a

Internal Medicine, Hospital Universitario de Gran Canaria Dr Negrin, Las Palmas de Gran Canaria, Spain b Neurology, Hospital Universitario de Gran Canaria Dr Negrin, Las Palmas de Gran Canaria, Spain Introduction: Behcet's disease is a chronic inflammatory syndrome, characterized by painful canker sores and ulcers in the mouth, genitals and/or eye. Other major symptoms are arthritis, meningitis,