238 32nd World Congress of Internal Medicine (October 24-28, 2014) WCIM 2014
PS 0731 Rheumatology
Miller Fisher Syndrome Secondary to Primary Sjögren’s Syndrome - Anti-CD 20 Responsive
Helena Lobo MARTINS1, Tomás Abrantes DA FONSECA2, Teresa SEQUEIRA2, Isabel ALMEIDA2, António MARINHO2, Carlos VASCONCELOS2
Centro Hospitalar Do Porto / Centro Hospitalar Do Baixo Vouga, Portugal1, Centro Hospitalar Do Porto, Portugal2
Neuropathy seems to be the most specific neurological complication of primary Sjögren’s syndrome (SS). Miller-Fisher syndrome (MFS) characterized by acute onset of ataxia, external ophthalmoplegia and arefl exia, is considered a variant form of Guillain Barre syndrome (GBS). GBS is linked to SS by case reports only. We present a case of 70-year-old woman with classifi cation criteria for primary SS: dry syndrome (xeros- tomia and xerophthalmia; Schirmer test = 5, Normal > 10), biopsy and scintigraphy of salivary glands compatible with SS, elevated SSa/Ro autoantibodies and polyclonal hypergammaglobulinemia. In 2007, presents paresthesias of both feet, takes Gabap- entin with poor response. In March 2009, admitted with diplopia and diffi culty in gait by ataxia; physical examination showed decrease in pain and vibratory hypoesthesia of the lower limbs (LL) with absence of osteo-tendon refl exes; lumbar puncture with increased protein (0.63) without cells; treated with intravenous Immunoglobulins (IV Ig) with signifi cant clinical improvement, except paresthesias in both feet. In the last three years, LL paresthesias worsened and spreaded to legs bilaterally under IV Ig, with relapse of diplopia and severe ataxia when extending dosage interval of IV Ig.
Electromyography and nerve biopsy inconsistent, performed under IV Ig. Thus, SS with neurological involvement characterized by diplopia, severe gait ataxia, severe sensory polyneuropathy with absent vibratory sensibilities and arefl exia – MFS like. Refractory and dependent of IV Ig treatment. Performed two perfusions of Rituximab 1 gram in December and January 2014, without new episodes of diplopia and ataxia and no further use of IV Ig until July 2014. This patient demonstrated clinical improvement after high dose of IV Ig, although dose dependent and refractory to treatment in the last three years. However, when the underlying disease was treated with anti-CD 20 therapy a complete response was observed.
PS 0733 Rheumatology
Acute Generalized Exanthematous Pustulosis Induced by Hydroxychloroquine in Patient with Sjögren’s Syn- drome
Chuiyong PAK1, Ji Seon OH1, Jin Hyoung KIM1, Kyung Hye PARK1, Jong Min LEE1, Seung Won CHOI1, Young Min KIM2
Department of Internal Medicine, Ulsan University Hospital, Korea1, Department of Pathology, Ulsan University Hospital, Korea2
Introduction: Acute generalized exanthematous pustulosis (AGEP) is a rare and se- vere cutaneous lesion that is usually provoked by drugs or infections. It characterized by acute onset of extensive, non-follicular, sterile pustular eruptions with fever and leukocytosis. Hydroxychloroquine is an antimalarial drug that has been widely used to treat rheumatic or dermatologic diseases. It has been reported as a rare cause of AGEP.
Case Summary: A 32-year-old woman with Sjögren’s syndrome was treated with hydroxychloroquine. Ten days after taking hydroxychloroquine, the patient developed fever and widespread erythematous pustular eruption on the trunk and extremities.
There was no mucous membrane involvement. The patient’s temperature was 39.4°C.
Laboratory test revealed leukocytosis (WBC 27,480/μL, with 83% neutrophils), and an elevated CRP (6.8mg/dL). Skin biopsy specimens showed acanthosis with subcorneal spongiform pustules and perivascular infi ltration of neutrophils in the dermis. Hydrox- ychloroquine was immediately withdrawn, and she was treated with systemic steroids, antihistamines and supportive measures. The cutaneous lesions resolved by the 20th day of the treatment.
Conclusion: We report a case of AGEP following administration of hydroxychloro- quine in patient with Sjögren’s syndrome. Hydroxychloroquine is a commonly used in rheumatologic conditions. Therefore, physician should keep in mind that it may cause severe and extensive cutaneous reactions.
PS 0734 Rheumatology
Bone Morphogenetic Protein 6 Polymorphisms are Associated with Radiographic Progression in Ankylosing Spondylitis
Young Bin Joo1, So-Young Bang1, Seunghun Lee1, Kyung Bin Joo1, Tae-Hwan Kim1 Hanyang University Hospital for Rheumatic Diseases, Korea1
Background: Nearly 25 genetic loci associated with susceptibility to ankylosing spondylitis (AS) have been identified by several large studies. However, there have been limited studies to identify the genes associated with radiographic severity of the disease. Thus we investigated which genes involved in bone formation pathways might be associated with radiographic severity in AS.
Methods: A total of 417 Korean AS patients were classifi ed into two groups based on the radiographic severity as defi ned by the modifi ed Stoke’ Ankylosing Spondylitis Spinal Score (mSASSS) system. Severe AS was defi ned by the presence of syndesmo- phytes and/or fusion in the lumbar or cervical spine (n=195). Mild AS was defi ned by the absence of any syndesmophyte or fusion (n=170). A total of 251 single nucleotide polymorphisms (SNPs) within 52 genes related to bone formation were selected and genotyped. Odds ratios (OR) and 95% confi dence interval (95% CI) were analysed by multivariate logistic regression controlling for age at onset of symptoms, sex, disease duration, and smoking status as covariates.
Results: We identifi ed new loci of bone morphogenetic protein 6 (BMP6) associated with radiographic severity in patients with AS that passed false discovery rate thresh- old. Two SNPs in BMP6 were signifi cantly associated with radiologic severity [rs270378 (OR 1.97, p = 6.74 x 10-4) and rs1235192 [OR 1.92, p = 1.17 x 10-3]) adjusted by covariates.
Conclusions: This is the first study to demonstrate that BMP6 is associated with radiographic severity in AS, supporting the role wingless-type like/BMP pathway on radiographic progression in AS.
PS 0735 Rheumatology
Chronic Periodontitis is Associated with Spinal Mobility and an Improvement after Periodontal Therapy is More Likely to Occur in Patients with Ankylosing Spondylitis
Eun Ha KANG1, Jung Tae LEE2, Hyo-Jeong LEE2, Joo Youn LEE3, Sung Hae CHANG4, Hyun Jung CHO1, Byoong Yong CHOI5, You-Jung HA1, Kyoung Un PARK6, Yeong Wook SONG7, Yun Jong LEE7
Seoul National University Bundang Hospital, Korea1, Seoul National University Bundang Hospital, Ko- rea2, Seoul National University Hospital, Korea3, Soonchunhyang University Cheonan Hospital, Korea4, Seoul Medical Center Public Corporation, Korea5, Seoul National University Bundang Hospital, Korea6, Seoul National University College of Medicine, Korea7
Objectives: To examine the associations between chronic periodontitis (CP) and an- kylosing spondylitis (AS) or between CP and clinical features of AS, and the effect of anti-TNFa therapy on periodontal treatment in Korean patients with AS.
Methods: 84 AS patients and 84 age- and sex-matched healthy controls were en- rolled. Clinical manifestations, disease activity and mobility parameters of AS, and periodontal parameters including probing pocket depth (PPD), clinical attachment loss (CAL), plaque index (PI), bleeding on probing (BOP) were assessed at baseline. Those with 4mm=PPD<7 mm on any tooth site had additional examinations at weeks 12 or 24 after periodontal treatment. Potential risk factors for CP including P. gingivalis infection were sought and adjusted.
Results: No associations were found between CP and AS or clinical phenotypes of AS. No difference was found in PPD, CAL, PI, BOP, P. gingivalis carrier frequencies, or anti-P. gingivalis titers between patients and controls at baseline. No correlation was found between disease activities and periodontal parameters. However, those with CP showed decreased chest expansion and increased BASMI scores in univariate analysis with the latter being signifi cant in multivariate analysis. Both patients and controls showed improvement in PPD and CAL after periodontal treatment, irrespective of anti-TNFa therapy. The improvement was independently associated with AS group, P.
gingivalis carriage, and baseline PPD>2.6 mm.
Conclusions: we could neither fi nd any association between CP and AS nor any cor- relation between disease activities and periodontal parameters. CP was not associated with any clinical phenotypes of AS, except for decreased mobility. Periodontal param- eters improved after treatment in both patients and controls but more frequently in patients. Anti-TNFa therapy was not harmful to AS patients who had conservative periodontal treatment.
