Abstracts from 8th Congress of the European Federation of Internal Medicine / European Journal of Internal Medicine 20S (2009), S1–S283 S153

P0455AUTOIMMUNE FIBROUS MASTOPATHY: ANALYSIS OF ONE CASEAND REVIEW OF THE LITERATURE

Saidane Zohra 1, Hammami Sonia 2, Njim Leila 1, Denguesli Walid 1,Mahjoub Silvia 2, Sakouhi Mohamed 1. 1Department of Gynecology;2Department of Internal Medicine

Autoimmune fibrous mastopathy or diabetic mastopathy is a rare entitydescription recent and little known.Habitullement, it occurs in patients followed for type 1 diabetes, and itwith the characteristic to simulate malignant as well on the clinical level asradiological.To ignore this benign pathology pseudotumorale, risks to lead a surgicaltreatment abusive.We to this end report a new case of fibrous mastopathy at a 34 year old youngwoman, this women is not known diabetic but she is carring an autoimmunethyroidite with vitiligo.

P0456SCLERODERMA

Claudia Borges, Catarina Pereira, Antonio Antunes, João Santos,Helena Carrondo, Odete Gomes, Celio Fernandes. Hospital Santo André-Epe-leiria Serviço Medicina Ii

Scleroderma is a multisystem disorder charaterized by skin thickening andvascular abnormalities.Is a rare disease, aproximately 80% of patients arefemales, and one- half present before the age 40.The authors present a case of a systemic sclerosis in a 74-year old female whowas sent to an internal medicine consult for persistent Raynaud phenomenonrefractory to previous medical treatment.Physical examination identified ulcerated lesions at the fingertips with skinthickening. Laboratory data was positive for antinuclear (ANA) and anti-SCL70 antibodies.Schirmer’s test was positive; thoracic computed tomography (CT) scanshowed ground glass pattern affecting both lungs; pulmonary function testswere normal; echocardiogram measured a pulmonary artery wedge pressureof 31 mmHG.Capillaroscopy was consistent with scleroderma.Prednisone, venodilators and penicillamine were started and although thelatter had to be discontinued due to an allergic reaction, significant clinicalimprovements have been registered one year after diagnosis.

P0457ROWELL SYNDROME – CASE REPORT

Ana Rita Estriga 1, Joana Coelho 2, Ana Ferreira 2, Maria Jose Serra 1.1Medicine Department 3, Hospital De Santo Antonio Dos Capuchos, Lisbon– Portugal; 2Dermatology Department, Hospital De Santo Antonio DosCapuchos, Lisbon – Portugal

Rowell syndrome consists in erythema multiforme-like lesions associated withlupus erythematosus (SLE), with positive antinuclear antibody, and frequentlyaccompanied by perniosis, positive anti-La (SS-B) or anti-Ro (SS-A) antibodyand reactive rheumatoid factor.The authors report the case of a 30-year-old woman, with no previous historyof SLE, presenting with perniosis and symmetric arthralgia (hands, feet andknees), with negative antinuclear antibodies. The patient developed malar rashand, later on, disseminated erythema multiforme-like lesions. Therapy withsteroids and immunoglobulin was started, with progressive remission of skinlesions.The authors emphasize the need of cooperation between internal medicine anddermatology in the early diagnosis, treatment, and follow-up of patients withboth systemic manifestations and coetaneous lesions.

P0458POLYMYOSITIS WITH PULMONARY INVOLVEMENT – CASEREPORT

Ana Rita Estriga, Fatima Viegas, Maria Jose Serra, Antonio Santos Castro.Medicine Department 3, Hospital De Santo Antonio Dos Capuchos, Lisbon –Portugal

Polymyositis is an idiopathic inflammatory myopathy which presents withsymmetric proximal muscular weakness and elevated serum muscle enzyme

levels, with electromyographic evidence of myopathic abnormalities, andcharacteristic findings at muscle biopsy. Extra-muscular manifestations mayappear in some cases, sometimes before muscular symptoms.The authors report the case of a 31-year-old woman, presenting with in-terstitial lung disease requiring long-duration oxygen treatment, with lateronset of symmetric proximal muscular weakness and elevated serum muscleenzyme levels. Electromyography and muscle biopsy confirmed the diagnosisof polymyositis.The authors emphasize the importance of interpreting the patients as a whole,remembering that the same disease can present differently over time, allowingthe reformulation of the diagnosis in light of new symptoms.

P0459MEDICAL DISORDERS ASSOCIATED WITH POSITIVEANTINEUTROPHIL CYTOPLASMIC ANTIBODIES:IMMUNOFLUORESCENCE PATTERN AND ENZYME-LINKEDIMMUNOSORBENT ASSAY

Chamaida Plasencia Rodríguez, Mariana Bastos Oreiro, Mónica LópezRodríguez, Miriam Estébanez Muñoz, Dora Pascual, Eugenio De MiguelMendieta. Hospital Universitario La Paz

Background: Antineutrophil cytoplasmic antibodies (ANCAs) are importantserological diagnostic markers for primary systemic small-vessel vasculiticdisorders as Wegener’s granulomatosis (WG), microscopic polyangiitis (MP),Churg-Strauss syndrome (CSS) and other systemic disorders. There are twotypes of laboratory techniques to test ANCAs: indirect immunofluorescenceassay (IIF) and Enzyme-linked immunosorbent assay (ELISA).Aims: Our aims were to identify positive ANCAs and its correlation withmedical disorders, taking into account the immunofluorescence pattern andELISA results.Methods: Positive serological ANCAs results, in two years, were obtainedfrom the database of the immunology laboratory of “La Paz” UniversityHospital, Madrid, Spain. IIF and ELISA assays were used for the detectionof ANCAs. Medical information was retrieved from the clinical records.Statistical analysis was performed with the software SPSS15.0Results: We identified 176 cases of positive ANCA; 18.2% were p-ANCA,14.8% c-ANCA, 57.4% x-ANCA and 9.7% were negative (positive ELISA,negative IIF). Of positive p-ANCA, 34.3% had been diagnosed with MP,6.3% with CSS, 21.9% with renal-limited vasculitis, 6.3% with unclassifiedvasculitis, 3.1% with ulcerative colitis (UC), and 28.1% with other diseases:autoimmune cholangitis, Raynaud disease, malignancies and systemic lupuserythematosus (SLE).Of positive C-ANCA, 42.3% had WG, 15.4% UC,11.5% unclassified vasculitis, 7.7% Crohn’s disease (CD), 3.8% MP and19.2% others: IgA nephropathy, extracapilar glomerulonephritis and infec-tions. Of positive x-ANCA, 57.4% had UC, 16.84% CD, 15.2% IID, and10.56% others: unclassified vasculitis, bronchial asthma, Behçet’s diseaseand celiac disease.MPO was positive in 93.8% of p-ANCA and 15.7% ofc-ANCA. PR3 was positive in 6.2 of p-ANCA and 80.5% of c-ANCA. PR3and MPO were both negative in 3.8% of the c-ANCA. ELISA for PR3 waspositive in 34 cases. Of these, 29.4% have been diagnosed with WG, 14.7%with UC, 5.9% with renal-limited vasculitis, 5.9% with unclassified vasculitis,5.9% with CD and 38.2% with others: deep vein thrombosis, SLE, Sjögrensyndrome, overlap syndrome, non-filiated gastrointestinal inflammatory dis-order and autoimmune cholangitis. MPO was positive in 44 cases. Of these,29.5% were diagnosed with MP, 18.2% with renal-limited vasculitis, 9.1%with unclassified vasculitis, 4.5% with CSS and 38.7% with others: extra-capilar glomerulonephritis, IgA nephropathy, granulomatous disease, silicosis,Raynaud disease, SLE and UC. The positive predictive value of ANCA for dediagnosis of vasculitis was 63.8%.Conclusion: ANCA testing currently plays a significant role in the diagnosisand classification of vasculitides. However, as shown in our study, there aremany non-vasculitic disorders that express positive ANCAs. For this reasonhistological confirmation should be obtained before committing patients totreatment with potentially toxic therapies.

P0460THE RARE INVOLVEMENT REGION OF SARCOIDOSIS:NASOPHARYNX

Safak Akın 1, Serkan Akın 1, Umut Kalyoncu 2, Omer Karadag 2. 1HacettepeUniversity, Internal Medicine; 2Hacettepe University, Internal Medicine,Rheumatology Division

Introduction: Sarcoidosis is a chronic, multisystemic, noncaseating granu-