SSA Antibodies Research Articles

Recommendations of diagnosis and treatment of primary Sjögren's syndrome in China

Sjögren's syndrome is a chronic systemic autoimmune disease characterized by lymphocyte proliferation and progressive exocrine gland damage. In China, standardized diagnosis and treatment for Sjögren's syndrome lags behind other common rheumatic diseases, such as rheumatoid arthritis and systemic lupus erythematosus. Based on the evidence and guidelines from China and other countries, Chinese Sjögren's Syndrome Collaborative Research Group together with stomatologist and ophthalmologist developed Standardization of diagnosis and treatment of primary Sjögren's syndrome. The purposes are: (1) to standardize the detection and interpretation of key indicators for the diagnosis of Sjögren's syndrome, including serum anti SSA antibody and labial gland pathology; (2) to suggest using widely accepted disease activity index in evaluation of the disease; (3) to standardize rational management for Sjögren's syndrome patients with topical and systemic diseases.

EP26.04: Fetal lupus erythematosus (FLE): a clinical spectrum caused by maternal SSA and SSB antibodies – report of two cases and review of literature

EP26.04: Fetal lupus erythematosus (FLE): a clinical spectrum caused by maternal SSA and SSB antibodies – report of two cases and review of literature

Systemic Scleroderma or Paraneoplastic Syndrome?

Abstract Introduction. Inflammatory rheumatic diseases are sometimes the first manifestation of neoplasia. Case presentation. A 57 year old female patient, heavy smoker, is admitted to our clinic for 2 days old pulp necrosis of the third finger, right hand, with onset of ischemia 1 week before admission. Other manifestations were Raynaud’s phenomenon, dry mouth, dysphagia and shortness of breath after moderate physical activity with a duration of about 6 months. She was repeatedly followed-up by a pulmonologist with chest computer tomography, which detected a dimensional evolutionary mediastinal lymph node, but not investigated further. The patient had skin thickening of the hands and face, microstomia and an area of dry necrosis of 1.5/1cm in the pulp of the third finger, right hand. Laboratory tests show the presence of antinuclear antibodies with positive anti SS-A antibodies and elevated levels of serum inflammatory markers. Vascular surgery examination excludes thromboangiitis obliterans or other large vessel diseases. The presumptive diagnosis is undifferentiated connective tissue disease with systemic sclerosis and Sjögren syndrome elements, most likely secondary to the mediastinal tumor. Mediastinal biopsy shows a heterogeneous metastasis of unknown origin, and further investigations: immunohistochemistry, multiple gene mutations tests, computed tomography scan of the chest, abdomen, and pelvis, mammography, Papanicolaou test and positron emission tomography scan did not identify the primary tumor, thus delaying oncologic treatment. Conclusion. Case of undifferentiated connective tissue disease with elements of systemic sclerosis and Sjögren syndrome, most probably paraneoplastic, with chest metastasis of unknown origin, in which diagnosis and treatment were delayed due to lack of primary tumor identification.

Unique Characteristics of Prepubertal Onset Systemic Lupus Erythematosus.

Objectives The aim of this study was to investigate the influence of age at disease onset on disease expression and outcomes of pediatric systemic lupus erythematosus SLE (pSLE). Methods A total of 103 patients with pSLE from Sultan Qaboos University Hospital, Oman, were retrospectively studied. Epidemiological, clinical phenotype, disease severity, serology, treatment, and outcome were compared among the three groups using univariate statistical tests. Results The mean disease duration of the cohort was 9.8 ± 4.7 years. The patients were divided into three groups: prepubertal onset (n=39) with mean age at diagnosis of 5.1 ± 2.0 years and pubertal disease onset (n=29) with mean age at diagnosis of 10.8 ± 1.0 years as well as postpubertal disease onset (n=35) group with mean age at diagnosis of 15.3 ± 1.6 years. The prepubertal pSLE cohort demonstrates unique characteristics with increased frequency of familial SLE (61%) of which 49% were from first-degree relatives. Similarly, this group had distinctive clinical features, which included increased renal disease in pubertal and postpubertal groups, respectively (51% vs 23% vs 20%; p=0.039). Prepubertal, similar to pubertal group, had a higher incidence of cutaneous manifestations than in the postpubertal group (74% vs 69% vs 46%; p=0.029). Laboratory features in prepubertal group were distinct with increased frequency of positive anti-cardiolipin antibodies (47%), anti-glycoprotein antibodies (42%), ANCA (62%), and low complement levels (97%) compared to pubertal and postpubertal group. The prepubertal group also has the lowest frequency of positive SSA antibodies (18%) and SSB antibodies (5.1%). The overall mean SLEDAI score in pSLE cohort was 15.6 ± 18.5. The mean SLEDAI scores among the groups showed no significance difference (p=0.110). The overall SLICC DI ≥1 was 36% with a mean damage score of 0.76 ± 1.38. No significant differences in damage index (SLICC DI ≥1) were noted among the groups. Conclusions Distinct clinical features were identified in prepubertal onset pSLE population of Arab ethnicity. Given the high rate of consanguineous marriage and high frequency of familial SLE in this cohort, these manifestations could be explained by higher frequency of genetic factors that influence the disease pathogenesis.

Two subgroups in systemic lupus erythematosus with features of antiphospholipid or Sj\xf6gren\u2019s syndrome differ in molecular signatures and treatment perspectives

BackgroundPrevious studies and own clinical observations of patients with systemic lupus erythematosus (SLE) suggest that SLE harbors distinct immunophenotypes. This heterogeneity might result in differences in response to treatment in different subgroups and obstruct clinical trials. Our aim was to understand how SLE subgroups may differ regarding underlying pathophysiology and characteristic biomarkers.MethodsIn a cross-sectional study, including 378 well-characterized SLE patients and 316 individually matched population controls, we defined subgroups based on the patients’ autoantibody profile at inclusion. We selected a core of an antiphospholipid syndrome-like SLE (aPL+ group; positive in the lupus anticoagulant (LA) test and negative for all three of SSA (Ro52 and Ro60) and SSB antibodies) and a Sjögren’s syndrome-like SLE (SSA/SSB+ group; positive for all three of SSA (Ro52 and Ro60) and SSB antibodies but negative in the LA test). We applied affinity-based proteomics, targeting 281 proteins, together with well-established clinical biomarkers and complementary immunoassays to explore the difference between the two predefined SLE subgroups.ResultsThe aPL+ group comprised 66 and the SSA/SSB+ group 63 patients. The protein with the highest prediction power (receiver operating characteristic (ROC) area under the curve = 0.89) for separating the aPL+ and SSA/SSB+ SLE subgroups was integrin beta-1 (ITGB1), with higher levels present in the SSA/SSB+ subgroup. Proteins with the lowest p values comparing the two SLE subgroups were ITGB1, SLC13A3, and CERS5. These three proteins, rheumatoid factor, and immunoglobulin G (IgG) were all increased in the SSA/SSB+ subgroup. This subgroup was also characterized by a possible activation of the interferon system as measured by high KRT7, TYK2, and ETV7 in plasma. In the aPL+ subgroup, complement activation was more pronounced together with several biomarkers associated with systemic inflammation (fibrinogen, α-1 antitrypsin, neutrophils, and triglycerides).ConclusionsOur observations indicate underlying pathogenic differences between the SSA/SSB+ and the aPL+ SLE subgroups, suggesting that the SSA/SSB+ subgroup may benefit from IFN-blocking therapies while the aPL+ subgroup is more likely to have an effect from drugs targeting the complement system. Stratifying SLE patients based on an autoantibody profile could be a way forward to understand underlying pathophysiology and to improve selection of patients for clinical trials of targeted treatments.

Recurrent aseptic meningitis- a rare clinical presentation of Sjogrens syndrome

Sjogren's syndrome most commonly presents with dry eyes, dry mouth, joint pain and fatigue. However, recurrent aseptic meningitis, reported as the most uncommon initial symptom, was the presenting feature in our case. We present the case of a 19-year-old female with recurrent episodes of aseptic meningitis. She presented with fever, headache, vomiting and photophobia. Neurological examination showed neck stiffness. Fundoscopy was normal. On two previous occasions her cerebrospinal fluid analysis was consistent with meningitis; however, it was normal at this presentation. Review of system revealed history of fatigue and sicca symptoms since early childhood. Autoimmune workup showed antinuclear antibodies with a titer of 1:400 and positive anti SSA (Ro) antibodies that led to the diagnosis of Sjogren's syndrome. She responded well to intravenous steroids, followed by oral prednisolone and hydroxychloroquine. To conclude, diagnosis of Sjogren's syndrome may also be considered in a patient presenting with recurrent aseptic meningitis.

Fetal Sinus Bradycardia Is Associated with Congenital Hypothyroidism: An Infant with Ectopic Thyroid Tissue.

Hypothyroidism is rarely included in the differential diagnosis for fetal sinus bradycardia. We report an infant with congenital hypothyroidism caused by ectopic thyroid tissue, who showed antenatal bradycardia. The baseline fetal heart rate was 100-110 bpm at 30 weeks of gestation, and fetal echocardiography revealed sinus bradycardia but no cardiac anomalies. Maternal thyroid function was normal (thyroid-stimulating hormone [TSH] 2.03 μIU/ml, free T3 2.65 pg/ml, and free T4 0.99 ng/dl) when measured at 31 weeks of gestation. Her serum anti SS-A and SS-B antibodies, anti-thyroglobulin, and microsomal antibodies were negative. A male infant without cardiac anomalies was delivered at 35 weeks and 4 days of gestation and admitted for prematurity and respiratory distress syndrome. The infant's heart rate was 70-110 bpm (normal: 120-160 bpm) on admission. On 8 days of age, thyroid function tests revealed that the infant had severe hypothyroidism (TSH 903.3 μIU/ml, free T3 1.05 pg/ml, and free T4 0.26 ng/dl). The prolonged jaundice assumed to be due to hypothyroidism. Oral levothyroxine sodium hydrate (10 μg/kg/day) was immediately started on day 8. After the treatment, the heart rate was gradually increased to 130-140 bpm as the infant's thyroid function was improved (TSH 79.8 μIU/ml, free T3 2.95 pg/dl, and free T4 1.66 ng/dl on day 22). The infant was diagnosed ectopic thyroid tissue because of the high thyroglobulin level (85.9 μg/l). In conclusion, congenital hypothyroidism should be included in the differential diagnosis in cases of fetal bradycardia without cardiac anomalies or maternal autoimmune diseases.

Detection of SSA and SSB Antibodies Associated with Primary Sjögren's Syndrome Using Enzyme-Linked Immunosorbent Assay.

Antibodies to Ro52/Ro60 (SSA) and La (SSB) are strongly associated to the autoimmune disease primary Sjögren's syndrome and are important in the serologic diagnosis of the disease. Several methods for detection of these antibodies exist such as indirect immunofluorescence, commercial western blot kits, in-house assays, and numerous commercial enzyme-linked immunosorbent assays (ELISAs). Dependent on the type of assay, sensitivity and specificity may vary notably. Especially ELISAs, where the antibody reactivity to synthetic peptides, recombinant or native proteins are determined, are often applied. This chapter describes detection of SSA and SSB antibodies by ELISA.

P13.04: Echo AV interval overestimates MCG PR interval in SSA+ pregnancies: evidence for new criteria for 1° AV block

We compared simultaneous fetal echo AV intervals (E-AV) and magentocardiogram (fMCG) PR intervals (M-PR) in SSA+ and control pregnancies to determine if an AV interval of + 2 z-scores correlated with a prolonged M-PR interval and 1° AV block. We searched the database of the University of Wisconsin Biomagnetism laboratory for pregnant women referred for fMCG with SSA antibodies. Maternal and pregnancy data were obtained from the mother's medical record. Fetal MCGs were performed using a 37-channel biomagnetometer (Magnes, 4D Neuroimaging, Inc). During the same session, we measured and averaged PR and cardiac RR intervals from 5 rhythm tracings and compared results to Echo Doppler AV interval (mitral inflow/aortic outflow) and RR intervals. Results were normalised for RR interval and compared by paired t-test. Regression analysis was used to test associations between gestational age (GA) and cardiac intervals. The mean M-PR was 23.3 ms less than the mean E-AV (95% CI: 20.1,26.5, p<0.001); this statistically significant difference persisted when normalised for RR interval. There were no significant differences between SSA and controls for RR, PR, or AV intervals. Neither E-RR interval or GA were associated with predicting E-AV; nor were M-RR or GA associated with predicting M-PR. However, the interaction between E-AV interval and GA were significantly associated with the AV-PR difference of 23.3 ms. Our results suggests that E-AV overestimates M-PR in both SSA and controls. Using the previous definition of 1° AVB (an AV interval of 150 ms) may result in unnecessary treatment of SSA+ normal fetuses. Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.

A CASE OF NEWLY DIAGNOSED RHEUMATOID ARTHRITIS IN AN OCTOGENARIAN PRECEDED BY A 10-YEAR HISTORY OF BRONCHIECTASIS AND MULTIPLE DVTS

SESSION TITLE: Lung Pathology 1 SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/09/2018 01:15 PM - 02:15 PM INTRODUCTION: Bronchiectasis is a disease increasing in prevalence in the United States with an estimated 100,000 patients affected. Common etiologies include cystic fibrosis, sequala of prior pulmonary infection, immune dysregulation and rheumatologic disorders. We present a case of rheumatoid arthritis diagnosed in an octogenarian, preceded by a 10-year history of progressive bronchiectasis and multiple DVTs. CASE PRESENTATION: A 79 year old male with history of upper and lower extremity DVTs, seborrheic dermatitis and pancytopenia was seen in clinic for evaluation of progressive bronchiectasis, first documented 10 years prior. Serial imaging revealed previously stable, mild, basal predominant disease (Fig. 1) with evidence of more rapid progression over the last 2 years (Fig. 2.) The patient remained essentially asymptomatic except for a dry cough. He was scheduled for diagnostic bronchoscopy, but before he could undergo the procedure, he presented to the ED with bilateral hand swelling and pain. Repeat ultrasound revealed increased propagation of his known upper extremity DVT while on Apixaban. There was increased concern at that time for underlying hypercoagulable state. Rheumatologic workup was undertaken, remarkable for elevated SS-A antibody, positive RF and ANA, negative CCP. The patient was treated with prednisone 15 mg daily which resulted in rapid symptom improvement. He then underwent bronchoscopy, which revealed normal cytology and cell differential and no growth on cultures. The patient was rapidly titrated down to 7.5 mg prednisone with continued resolution of his bilateral hand swelling. Repeat serologic testing showed him to have a persistent markedly elevated RF. At that time he was diagnosed with rheumatoid arthritis by 2010 ACR criteria. DISCUSSION: Extra-articular manifestations of rheumatoid arthritis are many, including VTE (2X increased risk) and pulmonary disease (up to 39-60% of patients.) Bronchiectasis is a less common pulmonary manifestation of the disease (2-3.1% prevalence in RA population.) In our patient, an evaluation for other causes of bronchiectasis was extensive and unrevealing. Bronchiectasis is much more commonly seen after the development of arthritis in RA, and is thought to result from activation of a common inflammatory pathway. In our patient, the reverse appears to be the case. He had a period of more rapid progression of bronchiectasis preceding the development of a new DVT and arthritis and finally leading to a diagnosis of RA. This pathophysiologic sequence may help explain the unusually advanced age at RA diagnosis (median age is 58.) CONCLUSIONS: Bronchiectasis is commonly associated with immune dysfunction. Rheumatoid arthritis is commonly associated with bronchiectasis, and the presence of bronchiectasis may act as a harbinger of RA, even many years before symptoms sufficient for diagnosis develop. Reference #1: Wilczynska M, Condliffe A, McKeon D. Coexistence of bronchiectasis and rheumatoid arthritis: revisited. Respir Care. 2013 Apr;58(4):694-701. Reference #2: Yunt Z, Solomon J. Lung disease in rheumatoid arthritis. Rheum Dis Clin North Am. 2015 May;41(2):225-36. Reference #3: Innala L et al. Age at onset determines severity and choice of treatment in early rheumatoid arthritis: a prospective study. Arthritis Res Ther. 2014 Apr 14;16(2):R94. DISCLOSURES: No relevant relationships by Andrew Hersh, source=Web Response No relevant relationships by Ian McInnis, source=Web Response Speaker/Speaker's Bureau relationship with Janssen Pharmaceuticals Please note: $1001 - $5000 Added 11/27/2017 by Michael Morris, source=Web Response, value=Consulting fee Speaker/Speaker's Bureau relationship with Vyaire Medical Please note: $1001 - $5000 Added 11/27/2017 by Michael Morris, source=Web Response, value=Consulting fee

Active NET formation in Libman–Sacks endocarditis without antiphospholipid antibodies: A dramatic onset of systemic lupus erythematosus

Although neutrophil extracellular traps (NETs) have been highlighted in several systemic inflammatory diseases, their clinical correlates and potential pathological role remain obscure. Herein, we describe a dramatic onset of systemic lupus erythematosus (SLE) with clear-cut pathogenic implications for neutrophils and NET formation in a young woman with cardiac (Libman–Sacks endocarditis) and central nervous system (psychosis and seizures) involvement. Despite extensive search, circulating antiphospholipid autoantibodies, a hallmark of Libman–Sacks endocarditis, could not be detected. Instead, we observed active NET formation in the tissue of the mitral valve, as well as in the circulation. Levels of NET remnants were significantly higher in serially obtained sera from the patient compared with sex-matched blood donors (p = .0011), and showed a non-significant but substantial correlation with blood neutrophil counts (r = 0.65, p = .16). The specific neutrophil elastase activity measured in serum seemed to be modulated by the provided immunosuppressive treatment. In addition, we found anti-Ro60/SSA antibodies in the cerebrospinal fluid of the patient but not NET remnants or increased elastase activity. This case illustrates that different disease mechanisms mediated via autoantibodies can occur simultaneously in SLE. NET formation with release of cytotoxic NET remnants is a candidate player in the pathogenesis of this non-canonical form of Libman–Sacks endocarditis occurring in the absence of traditional antiphospholipid autoantibodies. The case description includes longitudinal results with clinical follow-up data and a discussion of the potential roles of NETs in SLE.

Parameters of Somatosensory Evoked Potentials in Patients with Primary Sjӧgren's Syndrome: Preliminary Results.

Primary Sjogren's syndrome (pSS) is a chronic autoimmune disease. The aim of the study was to establish whether in patients with pSS without central nervous system (CNS) involvement, the function of the central portion of the sensory pathway can be challenged. In 33 patients with pSS without clinical features of CNS damage and normal head computed tomography scan, somatosensory evoked potentials (SEP) were studied. The results were compared to other clinical parameters of the disease, particularly to immunological status. The control group consisted of 20 healthy volunteers. Mean latency of all components of SEP was considerably prolonged in patients compared to the control group. Mean interpeak latency N20-N13 (duration of central conduction TT) did not differ significantly between the groups. However, in the study group, mean amplitude of N20P22 and N13P16 was significantly higher compared to healthy individuals. In patients with pSS, significant differences in SEP parameters depending on the duration of the disease and presence of SSA and SSB antibodies were noted. The authors confirmed CNS involvement often observed in patients with pSS. They also showed dysfunction of the central sensory neuron as a difference in the amplitude of cortical response, which indicates subclinical damage to the CNS.

CLINICAL SIGNIFICANCE OF SSA ANTIBODY IN PATIENTS WITH ARS ANTIBODY POSITIVE INTERSTITIAL PNEUMONIA

RespirologyVolume 22, Issue S3 p. 200-200 APSR Poster Abstracts CLINICAL SIGNIFICANCE OF SSA ANTIBODY IN PATIENTS WITH ARS ANTIBODY POSITIVE INTERSTITIAL PNEUMONIA First published: 23 November 2017 https://doi.org/10.1111/resp.13207_294Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat No abstract is available for this article. Volume22, IssueS3Supplement: 22nd Congress of the Asian Pacific Society of Respirology, International Convention Centre, Sydney, Australia, 23–26 November 2017November 2017Pages 200-200 RelatedInformation

Lymphocytic hidradenitis: A distinctive histopathological finding of annular erythema of Sjögren syndrome.

Lymphocytic hidradenitis is a non-specific histopathological feature observed in many dermatoses such as lupus erythematosus, morphea or scleroderma. When it occurs it is usually accompanied by the other distinctive histological features of those conditions. Isolated lymphocytic hidradenitis is uncommon and its clinical features and associated underlying medical conditions are still undetermined. We performed a retrospective review of patients who clinically presented with annular erythema between 2000 and 2016. Altogether, 30 patients with a histopathological presentation of isolated lymphocytic hidradenitis were identified. Their following characteristics were recorded: clinical features, number and localisation of lesions, serology and other associated medical conditions. Isolated lymphocytic hidradenitis was found most frequently in middle-aged women. Most patients (n=28, 93%) presented with many annular erythematous patches and plaques with mild pruritus; 22 (73%) had the SS-A antibody and 17 (57%) met the diagnostic criteria of Sjögren syndrome. Among these patients, 11 had primary and six had secondary Sjögren syndrome associated with systemic lupus erythematosus. Altogether 15 (50%) patients tested positive for a high titre of the antinuclear autoantibody. Other underlying diseases identified during the follow-up period include cryoglobulinaemia, angioimmunoblastic T-cell lymphoma, autoimmune hepatitis, hepatitis C infection and toxic thyroid goitre. Lymphocytic hidradenitis is a microscopic finding associated with annular erythemas of Sjögren syndrome. Systemic survey for sicca symptoms and work up for autoimmune diseases, including antinuclear antibodies, SS-A, SS-B antibodies, cryoglobulin, lymphoma, viral and autoimmune hepatitis should be performed to facilitate the correct diagnosis.

Coexistence of sarcoidosis and primary Sjögren syndrome: a clinical analysis and literature review

Four patients with coexistence of sarcoidosis and primary Sjögren syndrome (pSS) were retrospectively analyzed.All patients were female, who were referred to our department mainly because of respiratory symptoms.Positive antinuclear antibody(ANA) was detected in 2 patients and anti-Sjögrens syndrome A (SSA) antibody positive in 1 patient.All patients presented specific histologic patterns of both sarcoidosis and pSS.Publications related to coexistence of these two diseases were reviewed.Forty-one patients were finally included in the analysis, among whom 37 confirmed patients were from literature search.There were 37 women and 4 men.The main clinical features presentation were xerophthalmia in 40, xerostomia in 38, hilaradenopathies in 28, interstitial lung disease in 15, respiratory symptoms in 13.The main immunologic data were positive ANA in 23, SSA antibody in 19, anti-Sjögrens syndrome B antibody in 10 and rheumatoid factor in 12.All patients presented specific histologic patterns of both diseases.Patients with both sarcoidosis and pSS of ten represent multisystemic involvement and positive immunologic parameters, as well as the dual expression of specific histologic characteristics.

Diplopia and Sjogren's disease: A rare case report

Sjogren's syndrome is a chronic autoimmune disorder which affects the exocrine glands with lymphocytic infiltration, and occasionally involves central nervous system. It is usually rare and manifests as a lesion in the trigeminal nerve. Our case discusses the involvement of the oculomotor and abducens nerves along with the prevalence of such cases as seen on literature review. We describe a case of a middle aged woman who presented with ophthalmoplegic symptoms. The symptoms resolved in response to steroid therapy and serum analysis was positive for anti SSA antibodies. Increasing use of imaging modalities has enabled identifying cranial nerve enhancements easily. Correlating this to serum analysis, as in our case; has helped identify more cases of third and sixth cranial nerve involvement than was previously known to occur with primary Sjogren's syndrome.

Heart Failure - A Rare Initial Manifestation of Systemic Lupus Erythematosus

1.1 Context Cardiac involvement is a leading cause of death in systemic lupus erythematosus (SLE). Heart failure (HF) is a potentially life threatening complication of lupus myocarditis which is rarely described as an initial manifestation of SLE. We are presenting a case of HF as initial manifestation of SLE. 1.2 Case description 49 year old male with no past medical history presented with dyspnea and generalized weakness of 6 weeks duration. Physical exam was remarkable for tachycardia, S3 gallop, bibasilar crackles, and 2+ pitting edema. Echocardiogram showed ejection fraction of 35% without regional wall motion abnormalities or pericardial effusion. Cardiac catheterization showed normal coronaries. Serological tests indicated positive ANA, Anti-smooth muscle, SCL-70 and SSA antibodies. Patient was given methyl prednisone 1 g/day for 3 days followed by tapering dose of oral prednisone in addition to intravenous furosemide. He was discharged on prednisone 40 mg, hydroxychloroquine 200mg in addition to B-Blockers and ACE inhibitors. On follow up evaluation after 4 weeks there was complete resolution of his symptoms with improvement in ejection fraction to 45%. 1.3 Conclusion HF due to myocarditis can be a rare initial presenting manifestation of SLE. Identifying the cause is critical as it dictates therapy. Prompt recognition and a combination of supportive treatment and immunosuppression usually results in a favorable outcome.

Unusual presentation Of Sjögren-associated neuropathy with plasma cell-rich infiltrate.

Sjögren syndrome is thought to be a lymphocyte-driven process. Peripheral nervous system involvement occurs in about 20%-25% of patients. A sensory-predominant, large-fiber peripheral neuropathy is most common, and it is usually associated with a subacute to chronic presentation. We report a rare case of an acute Sjögren-associated, sensory predominant, length-dependent peripheral neuropathy mimicking Guillain-Barré syndrome. The patient presented with sensory ataxia preceded by fever and polyarthralgia. She gave a history of years of dry eyes and dry mouth. She had a positive Shirmer test, abnormal salivary gland scan, and positive SS-A and SS-B antibodies. A sural nerve biopsy showed an unusual, dense, non-IgG4, polyclonal, plasma-cell perivascular infiltrate. The patient responded to treatment with weekly pulse intravenous methylprednisolone. Sjögren syndrome can present with acute-onset, sensory predominant peripheral neuropathy. The role of plasma cells in Sjögren syndrome is unexplored and deserves further study. Muscle Nerve 55: 605-608, 2017.

Quiz Page March 2016: A 60-Year-Old Man With Fever, Night Sweats, and Acute Kidney Injury

Quiz Page March 2016: A 60-Year-Old Man With Fever, Night Sweats, and Acute Kidney Injury

Application of Various Statistical Models to Explore Gene-Gene Interactions in Folate, Xenobiotic, Toll-Like Receptor and STAT4 Pathways that Modulate Susceptibility to Systemic Lupus Erythematosus.

In view of our previous studies showing an independent association of genetic polymorphisms in folate, xenobiotic, and toll-like receptor (TLR) pathways with the risk for systemic lupus erythematosus (SLE), we have developed three statistical models to delineate complex gene-gene interactions between folate, xenobiotic, TLR, and signal transducer and activator of transcription 4 (STAT4) signaling pathways in association with the molecular pathophysiology of SLE. We developed additive, multifactor dimensionality reduction (MDR), and artificial neural network (ANN) models. The additive model, although the simplest, suggested a moderate predictability of 30 polymorphisms of these four pathways (area under the curve [AUC] 0.66). MDR analysis revealed significant gene-gene interactions among glutathione-S-transferase (GST)T1 and STAT4 (rs3821236 and rs7574865) polymorphisms, which account for moderate predictability of SLE. The MDR model for specific auto-antibodies revealed the importance of gene-gene interactions among cytochrome P450, family1, subfamily A, polypeptide 1 (CYP1A1) m1, catechol-O-methyltransferase (COMT) H108L, solute carrier family 19 (folate transporter), member 1 (SLC19A1) G80A, estrogen receptor 1 (ESR1), TLR5, 5-methyltetrahydrofolate-homocysteine methyltransferase reductase (MTRR), thymidylate synthase (TYMS). and STAT4 polymorphisms. The ANN model for disease prediction showed reasonably good predictability of SLE risk with 30 polymorphisms (AUC 0.76). These polymorphisms contribute towards the production of SSB and anti-dsDNA antibodies to the extent of 48 and 40%, respectively, while their contribution for the production of antiRNP, SSA, and anti-cardiolipin antibodies varies between 20 and 30%. The current study highlighted the importance of genetic polymorphisms in folate, xenobiotic, TLR, and STAT4 signaling pathways as moderate predictors of SLE risk and delineates the molecular pathophysiology associated with these single nucleotide polymorphisms (SNPs) by demonstrating their association with specific auto-antibody production.