Acute Systemic Lupus Erythematosus Research Articles

Abstract 16766: Increased Clinical and Financial Burden of Heart Failure Hospitalizations in Patients With Systemic Lupus Erythematosus: Insights From the National Inpatient Sample 2012-2014

Introduction: Systemic lupus erythematosus (SLE) is a chronic inflammatory disease associated with increased cardiovascular (CV) co-morbidities. Recent evidence suggests that (SLE) is associated with higher incidence of heart failure (HF) independent of traditional CV risk factors. Despite a known association with accelerated CV diseases, the association between burden of acute decompensated HF (ADHF) and SLE is not well-characterized. Therefore, we used a large national database to examine the clinical and financial burden of ADHF in SLE. Hypothesis: SLE would associate with increased hospitalization for ADHF along with a longer length of stay (LOS) and higher hospital charges. Methods: SLE patients (n=160,350) older than 18 years were identified in the Nationwide Inpatient Sample (NIS) 2012-2014 databases using the ICD-9 code 710.0. ADHF was classified using appropriate ICD-9 codes from 428.0-428.9. Demographics, traditional CV risk factors, and HF-related outcomes were defined in SLE patients and compared to the general population. Results: Patients in the general population and with SLE were middle-aged (median 56 and 59 years). Prevalence of hypertension and chronic kidney disease was higher in SLE patients, whereas diabetes, coronary artery disease, dyslipidemia was lower (Table 1). ADHF rate, LOS and total hospital charges were increased in SLE. Furthermore, LOS (standardized β=0.003, p<0.001) and hospital charges (β=0.006, p<0.001) were associated with SLE beyond CV risk factors. Finally, after adjustment for CV risk factors (age, sex, hypertension, diabetes, dyslipidemia, coronary artery disease, chronic kidney disease, smoking), dysrhythmias, thyroid disorders, alcohol use and median household income, SLE associated with ADHF (OR, 95% CI: 1.53 [1.51-1.55], p<0.001), increased LOS (1.14 [1.11-1.17], p<0.001) and increased hospital charges (1.12 [1.09-1.15], p<0.001) (Table 1). Conclusion: SLE is associated with increased clinical and financial burden of ADHF beyond CVRF, underscoring the need for greater early clinical awareness of SLE in HF patients. Larger prospective studies are needed to confirm our findings.

A Rare Presentation of Toxic Epidermal Necrolysis – Like Acute Systemic Lupus Erythematosus

A 25-year-old woman with a history of systemic lupus erythematosus (SLE) was transferred from an outside hospital with a worsening painful generalized rash and oral ulcerations for the prior 3 weeks due to concern for Stevens-Johnson Syndrome / toxic epidermal necrolysis (TEN). Exam revealed denuded erythematous plaques covering over 80% of the patient’s body surface area and a 4.2 x 2.6 cm ulcerated plaque of the superior hard palate. Histology demonstrated parakeratosis and compact hyperkeratosis overlying an atrophic epidermis with vacuolar interface change, prominent keratinocyte dyskeratosis, and a thickened basement membrane zone (BMZ). The superficial dermis had a mild predominantly lymphocytic perivascular infiltrate and superficial dermal mucin deposition. Direct immunofluorescence was positive for IgG (granular, BMZ), C3 (granular, BMZ), and IgA was negative. Labs were remarkable for positive ANA (1:160), ds-DNA, anti-Smith, anti-RNP, low C3/C4, with negative anti-SSA/SSB. The clinicopathological correlation was most consistent with the diagnosis of TEN-like acute systemic lupus erythematosus (TEN-like ASLE). Our patient improved with treatment for her ASLE. This case highlights a challenging clinicopathologic differential diagnosis.

Association of Acute Myeloid Leukemia and Systemic Lupus Erythematosus: A Case Report

Systemic lupus erythematosus is an autoimmune Inflammatory disease that can affect multiple systems and organs of the body including skin, kidneys, lungs, joints and nervous system.Some previous studies up light thatSLE was associated with increased risk of Acute leukemia (SIR = 2.3). The incidence ofthe association of AMLand SLE is not known but there are few case reports in literature. The aim of this report was to up light the difficulties wich occurred in the diagnosis and the management of acute myeloid leukemia in patient with systemic lupus erythematosus. Patient, 43 years old, diabetic treat withinsulin, wichsister is followed for Behcet's disease, was admited for AML with trisomy of chromosome 4 and 8 and thrombophlebitis of the superior sagittal sinus, Chest CT-Scan showed minimal bilateral pleural effusion. She was treated according to Morocco National protocol AML-MA-2011. CT-Scan was done atday 12 ofchemotherapy and noticedthe persistance of the minimal bilateral pleural effusion.At day 19 the patient presentedmalar rash, and right axillary adenitis. The biopsy of the adenitis show the presence of LE cells. According to internal physician recommendations we add steroids to the treatement. The cough and fever deasapear. The CT-scan for control was normal after two weeks of steroids. Patient is in complete remission after induction I. After a following-up of five months, the patient still well,but present severy infections during chemotherapy cycles and a bad tolerance for the treatement. The association SLE-AML is rare. The diagnosis of the association SLE-AML is difficult. It is management is also difficult according to commorbidity, severy infectious because of dicline of immunity, and less tolerance to AML chemotherapy.

Clonal evolution and antigen recognition of anti-nuclear antibodies in acute systemic lupus erythematosus

The evolutional process of disease-associated autoantibodies in systemic lupus erythematosus (SLE) remains to be established. Here we show intraclonal diversification and affinity maturation of anti-nuclear antibody (ANA)-producing B cells in SLE. We identified a panel of monoclonal ANAs recognizing nuclear antigens, such as double-stranded DNA (dsDNA) and ribonucleoproteins (RNPs) from acute SLE subjects. These ANAs had relatively few, but nonetheless critical mutations. High-throughput immunoglobulin sequencing of blood lymphocytes disclosed the existence of sizable ANA lineages shearing critical mutations intraclonally. We further focused on anti-DNA antibodies, which are capable to bind to both single-stranded (ss) and dsDNA at high affinity. Crystal structure and biochemical analysis confirmed a direct role of the mutations in the acquisition of DNA reactivity and also revealed that these anti-DNA antibodies recognized an unpaired region within DNA duplex. Our study unveils the unique properties of high-affinity anti-DNA antibodies that are generated through antigen-driven affinity maturation in acute phase of SLE.

Atypical Presenting Symptoms of Acute Onset Systemic Lupus Erythematosus with Enteritis and Cystitis.

Lupus enteritis and lupus cystitis are relatively rare manifestations of systemic lupus erythematosus. Some patients develop severe complications such as bowel perforation, infarction, obstruction, or irreversible bladder dysfunction. Early diagnosis is critical for management of lupus enteritis and cystitis. We report a 48-year-old Japanese man who presented with initial manifestations of abdominal pain, severe diarrhea, and bloody feces. The diagnosis was delayed due to atypical initial symptoms, resulting in clinical worsening. Physicians should be aware of typical computed tomography findings of lupus enteritis and lupus cystitis.

Diversity, cellular origin and autoreactivity of antibody-secreting cell population expansions in acute systemic lupus erythematosus.

Acute SLE courses with antibody-secreting cells (ASC) surges whose origin, diversity, and contribution to serum autoantibodies remain unknown. Deep sequencing, autoantibody proteome and single-cell analysis demonstrated highly diversified ASC punctuated by VH4-34 clones that produce dominant serum autoantibodies. A fraction of ASC clones contained unmutated autoantibodies, a finding consistent with differentiation outside the germinal centers. A substantial ASC segment derived from a distinct subset of newly activated naïve cells of significant clonality that persist in the circulation for several months. Thus, selection of SLE autoreactivities occurred during polyclonal activation with prolonged recruitment of recently activated naïve B cells. These findings shed light into SLE pathogenesis, help explain the benefit of anti-B cell agents and facilitate the design of future therapies.

Clinical characteristics and in-hospital outcome of patients with acute coronary syndromes and systemic lupus erythematosus

ObjectiveDue to the chronic inflammation associated with systemic lupus erythematosus (SLE), patients develop premature atherosclerosis and the disease is a risk factor for acute myocardial infarction. The best interventional treatment for acute coronary syndrome (ACS) in these patients is unclear. The objective of this study is to describe the baseline characteristics, clinical manifestations, treatment and in-hospital outcome of patients with SLE and ACS. MethodsEleven SLE patients with ACS were analyzed retrospectively between 2004 and 2011. The following data were obtained: age, gender, clinical and electrocardiographic characteristics, Killip class, risk factors for ACS, myocardial necrosis markers (CK-MB and troponin), creatinine clearance, left ventricular ejection fraction, inflammatory markers (C-reactive protein and erythrocyte sedimentation rate), drugs used during hospital stay, treatment (medical, percutaneous or surgical) and in-hospital outcome. The statistical analysis is presented in percentages and absolute values. ResultsTen of the patients (91%) were women. The median age was 47 years. Typical precordial pain was present in 91%. Around 73% had positive erythrocyte sedimentation rate. The vessel most often affected was the anterior descending artery, in 73%. One patient underwent coronary artery bypass grafting, seven underwent percutaneous coronary intervention with bare-metal stents and three were treated medically. In-hospital mortality was 18%. ConclusionsDespite the small number of patients, our findings were similar to those in the literature, showing coronary artery disease in young people with SLE due to premature atherosclerosis and a high mortality rate.

A Case of Lupus Pneumonitis Therapy in a Patient with Acute Systemic Lupus Erythematosus

Correspondence to Ji-Min Oh, M.D. Department of Internal Medicine, SAM Medical Center, 9 Samdug-ro, Manan-gu, Anyang 430-733, Korea Tel: +82-31-467-9226, Fax: +82-31-467-9198, E-mail: ninijang@naver.com Copyrightc 2014 The Korean Association of Internal Medicine This is an Open Access article distributed under the terms of the Creative Commons Attribution Non Commercial License (http://creativecommons.org/licenses/by nc/3.0/) which permits unrestricted noncommercial use, distribution, and reproduction in any medium, provided the original work is properly cited. 전신 홍반 루푸스 환자에게 발생한 급성 루푸스 폐렴 치료 1예

Coronary Artery Bypass Grafting in a Patient Initially Presenting with Systemic Lupus Erythematosus

We report a 51-year-old man who was diagnosed with concomitant coronary artery disease and systemic lupus erythematosus (SLE). He required urgent coronary artery bypass grafting (CABG) before the initiation of steroid therapy. Steroid therapy was initiated on postoperative day 2 due to the aggravation of SLE. However, he displayed persistent infection and fever, and the steroid dose was gradually decreased, resulting in the worsening of SLE by postoperative day 21. We closely monitored his infection status and renal function and regulated the steroid dose accordingly. The patient stabilized and was discharged on postoperative day 60 without further complication. Meticulous post-operative management is required in acute SLE patients who need open heart surgery.

A case of toxic epidermal necrolysis-like skin lesions with systemic lupus erythematosus and review of the literature

Subepidermal bullous lesions and toxic epidermal necrolysis-like (TEN-like) lesions can occur in patients with systemic lupus erythematosus (SLE). In this report, we describe a case of a patient previously diagnosed with SLE who experienced TEN-like skin lesions with unusual subacute progression in the context of the current literature. We present a recent case of TEN-like lupus erythematosus and review of studies published in English identifying SLE cases mimicking TEN, accessed via PubMed and Google Scholar databases. The keywords used in the search were: TEN, SLE, TEN-like SLE, and TEN-like lesions. The search covered all articles from January 1980 to November 2011. A 52-year-old male presented with fatigue, weakness, and weight loss (23 kg in two months). Skin redness started across nose and cheeks six months before admission. Bilateral pleural effusions were observed in a thorax tomography taken in the referral hospital two months prior to admission. Because of articular involvement, antinuclear antibody (ANA), and anti-dsDNA positivity, the patient was diagnosed with SLE. We initiated a punch skin biopsy, and the findings were consistent with Stevens-Johnson syndrome. There was marked basal layer necrosis in the epidermis, and there was predominantly lymphohistiocytic infiltrate in the dermis. A total of 22 cases, including our case, with TEN-like lupus erythematosus were reported in the literature. In addition, cutaneous lupus had positive ANAs in 18 of 22 patients (81.8%). The patients were aged 12 to 76 years; 21 cases were women and only one patient was male. Skin involvement, including the rare variant of TEN-like acute cutaneous SLE, is very common among SLE patients. The acute syndrome of pan-epidermolysis or apoptotic pan-epidermolysis may become a useful designation when considering a clinical diagnosis of drug-induced TEN or SLE. Further studies are required to verify our findings.

Markers of acute neuropsychiatric systemic lupus erythematosus: a multidisciplinary evaluation

This study was aimed to assess: (1) the additive diagnostic utility of diffusion-weighted imaging (DWI) and magnetic resonance angiography (MRA) over conventional MRI in detecting brain lesions in patients with acute primary neuropsychiatric systemic lupus erythematosus (NPSLE), and (2) the relevance of their findings to the associated NP manifestations. Included were 34 patients with acute NPSLE with mean age of 33.26 ± 10.14 years and duration of illness of 3.33 ± 1.71 years. Clinical interviewing and psychiatric and cognitive evaluations were performed by applying the criteria of the diagnostic and statistical manual of mental health disorders criteria (DSM-IV), Stanford Binet Subset Testing, Mini-Mental State Examination and Wechsler Memory Scale-Revised. Serologic tests included looking for antinuclear antibodies, anti-double strand DNA, anti-phospholipid antibodies. Radiologic evaluation included conventional MRI, DWI and MRA. One or more NP manifestations were diagnosed in 28 patients, in which cognitive deficits were reported with headache, psychosis and CVS. Anti-phospholipid antibodies were reported in patients with CVS. Twenty patients (71.43 %) with primary NPSLE (n = 28) had MRI abnormalities in which hyperintense signals at subcortical and periventricular white matter and at the junction between the gray and white matter represented 75 % (n = 15) and with headache (n = 6), psychosis (n = 6) and acute confusional state (n = 3) with and without cognitive deficits, respectively. Moderate-sized infarctions with restricted diffusion in the distribution of middle cerebral arteries were represented in 35 % (n = 7) and with CVS, of them, 71.43 % (n = 5) had beading and focal narrowing of carotid arteries were consistent with vasculitis. Brain atrophy represented 20 % (n = 4) and with psychosis. Compared to those with normal MRI, patients with MRI abnormalities were older (P < 0.050) and had longer duration of illness (P < 0.050). To conclude, although DWI and MRA are helping in more precise etiopathologic diagnosis compared to conventional MRI, but their relevance to the present NP manifestations is still limited.

Acute onset of systemic lupus erythematosus with extensive gastrointestinal and genitourinary involvement

Lupus enteritis may occur as an acute presentation of systemic lupus erythematosus (SLE), and is not uncommonly associated with acute uretero-cystitis and/or hydronephrosis. Extensive involvement of the gastrointestinal tract from the stomach to the rectum in acute SLE is uncommon. We describe a 22-year-old Chinese female who presented with acute extensive gastrointestinal and genitourinary involvement who recovered following pulse intravenous methylprednisolone and cyclophosphamide. A high index of clinical suspicion is required to arrive at the diagnosis of SLE where only acute gastrointestinal and genitourinary manifestations are predominant, and other clinical, haematological and biochemical features of SLE are absent.

Interferon-α stimulates TRAIL expression in human keratinocytes and peripheral blood mononuclear cells: implications for the pathogenesis of cutaneous lupus erythematosus

The tumour necrosis factor-related apoptosis-inducing ligand TRAIL has been shown to participate in the pathogenesis of systemic lupus erythematosus (SLE). The accumulation of apoptotic cell debris has been hypothesized to induce this autoimmune inflammation, and TRAIL may trigger this programmed cell death. Furthermore, TRAIL is among the interferon (IFN)-regulated genes which are typically expressed in the peripheral blood of patients with acute SLE. As an inappropriate activation of the type I IFN system plays an important role in both SLE and cutaneous lupus erythematosus (CLE) subsets, we hypothesized that TRAIL might also participate in the pathogenesis of CLE. Immunohistochemistry and immunofluorescence analyses were used to identify and localize TRAIL-expressing cells in CLE skin specimens. TRAIL expression in peripheral blood mononuclear cells (PBMC) isolated from patients with CLE was measured by flow cytometry. The impact of IFN-α treatment on TRAIL expression by keratinocytes and PBMC was evaluated by reverse transcription-polymerase chain reaction and flow cytometry. Keratinocytes are beside CD11c+ and BDCA2+ dendritic cells the major TRAIL-expressing cells in CLE lesions. TRAIL is upregulated on the surface of circulating CD11c+ PBMC isolated from patients with CLE. Treatment of keratinocytes and PBMC with recombinant IFN-α strongly enhances TRAIL expression by these cells. The proapoptotic TRAIL receptor R1 is expressed by keratinocytes in CLE skin lesions. TRAIL is strongly expressed in the skin and the blood of patients with CLE and may trigger the apoptotic death of kerationcytes in CLE via the TRAIL receptor R1. An IFN-α-induced TRAIL expression may in this way participate in the pathogenesis of CLE.

Posterior Reversible Encephalopathy Syndrome Presenting as Balint Syndrome

Balint syndrome is a disorder of inaccurate visually guided saccades, optic ataxia, and simultanagnosia that typically results from bilateral parieto-occipital lesions. Visual perception disturbances in the posterior reversible encephalopathy syndrome (PRES) include hemianopia, visual neglect, and cerebral blindness, but Balint syndrome had not been recognized. We report Balint syndrome associated with PRES in a 37-year-old woman with acute hypertension and systemic lupus erythematosus. Balint syndrome can be an initial presentation of PRES.

Acute systemic lupus erythematosus on the acute medical take: are we missing anything?

### History and examination A 42-year-old man with a background of systemic lupus erythematosus (SLE) presented acutely to the medical admissions unit (MAU) with pleuritic chest pain, shortness of breath and arthralgia. He was receiving no regular medications and denied any other medical problems,

Systemic lupus erythematosus (SLE) pneumonitis mimicking swine influenza pneumonia during the swine influenza (H1N1) pandemic

Background We present a young woman with a negative medical history who presented with acute systemic lupus erythematosus (SLE) pneumonitis mimicking swine influenza (H1N1) pneumonia. Because this case occurred during the H1N1 pandemic, the initial diagnostic impression was of H1N1 pneumonia. Methods Although her clinical and laboratory findings were consistent with the diagnosis of H1N1 pneumonia, e.g., fever, sore throat, dry cough, arthralgias, myalgias, thrombocytopenia, relative lymphopenia, and elevated serum transaminases, some findings suggested an alternate diagnosis, e.g., leukopenia, a highly elevated erythrocyte sedimentation rate, highly elevated serum ferritin levels, elevated antinuclear antibody (ANA) levels, and double-stranded (DS) DNA titers. Her chest x-ray showed an accentuation of basilar lung markings, with a small pleural effusion similar to the chest x-ray findings of early H1N1 pneumonia. Initially, her headaches were thought to be related to central nervous system manifestations of H1N1. Results After laboratory test results demonstrated elevated ANA and anti-DS DNA titers, she was diagnosed with acute SLE pneumonitis. The take-home lesson for clinicians is that other infectious diseases, e.g., human parainfluenza virus or Legionnaires’ disease, can mimic H1N1 pneumonia during an influenza pandemic. Excluding asthma, congestive heart failure, exacerbations of acute bronchitis, chronic obstructive pulmonary disorder, and pulmonary interstitial disease, noninfectious mimics of H1N1 are extremely rare. Conclusion To the best of our knowledge, this is the first reported case of de novo SLE pneumonitis mimicking H1N1 pneumonia during the swine influenza pandemic.

Parvovirus B19–associated Systemic Lupus Erythematosus: Clinical Mimicry or Autoimmune Induction?

To the Editor: Recent research has focused on the role of parvovirus B19 in the etiopathogenesis of systemic lupus erythematosus (SLE). The acute manifestations of B19 infection, including symmetric polyarthritis, cytopenia, and macular erythema, bear striking similarity to those found in SLE. Further, infection with B19 elicits autoantibodies to antigens commonly found in patients with SLE, including nuclear antigens, dsDNA, and phospholipids1. These symptoms and laboratory abnormalities are transient in some cases, yet persist in others, sparking debate over whether the similarities observed between acute B19 infection and SLE are mere coincidence, or whether the virus does in fact induce chronic autoimmunity. We describe a case of SLE possibly triggered by parvovirus B19, and we conducted a literature search to identify other cases in which B19 infection induced either transient or persistent SLE symptoms. To identify potential prognostic factors for the development of persistent disease, we performed a 2-tailed t test (p value for statistical significance = 0.05) to analyze differences in mean antinuclear antibody (ANA) titers in patients with transient versus persistent symptoms. A 59-year-old woman was referred for a history of urticarial plaques in the setting of fatigue, facial flushing, and polyarthritis of the hands and elbows. Her joint symptoms first developed 2 years ago; at the time, she was treated for presumed rheumatoid arthritis. However, she subsequently developed urticarial plaques on the torso, back, and arms. Biopsy of a plaque revealed an upper dermal angiocentric mixed polynuclear and mononuclear cell inflammatory infiltrate with … Address correspondence to M.T. Hession; E-mail: mth2114{at}columbia.edu

Hemophagocytic syndrome as one of the main primary manifestations in acute systemic lupus erythematosus - case report and literature review

Hemophagocytic syndrome is an unusual but fatal disorder characterized by pancytopenia and activation of macrophages. We describe one case of acute systemic lupus erythematosus with an unusual presentation of hemophagocytic syndrome not related to infection. The patient presented with pancytopenia related to increasing hemophagocytic activity of histiocytes in the bone marrow. Concomitant class IV World Health Organization lupus nephritis, serositis, high titer of antinuclear factor and positive test for anti-DNA antibody fitted the diagnostic criteria of systemic lupus erythematosus. She also presented with alveolar hemorrhage and lupus myocarditis. She underwent immunosuppressive therapy with recovery from the hemophagocytic syndrome. Therefore, diagnosis of acute lupus hemophagocytic syndrome was made. The clinical presentation, laboratory diagnosis, and management of the patient are discussed and the literature was reviewed and presented, with emphasis on a possible distinct lupus subset, which includes a more aggressive systemic disease with heart involvement.

Disease activity, damage and survival in Mexican patients with acute severe systemic lupus erythematosus

Systemic lupus erythematosus (SLE) is a clinical syndrome of varying severity. Although the survival and prognosis of SLE have steadily improved, there is a group of patients who present an acute fatal outcome despite aggressive therapy. We designed this study to evaluate the factors associated with mortality in patients with acute severe SLE. During 2004-06, 41 Mexican SLE patients that could not be managed in the out-patient clinic and with acute severe major organ system involvement [nephritis, severe thrombocytopenia (platelet count below 20 000 per microL) acute neuropsychiatric pulmonary, gastrointestinal or cardiac disease and generalized vasculitis] were studied. During the first admission, disease activity (SLE Disease Activity Index (SLEDAI), SLE Activity Measured), damage [SLE International Collaborating Clinics (SLICC)], and therapy were assessed. Survival using Kaplan-Meier curves, odd ratios with 95% confidence interval and logistic regression analysis were used to determine risk factors for mortality. Ninety percent were female with a mean age of 29 +/- 19 years and mean disease duration of 21 +/- 9 months. The principal causes of first admission were renal (27%), SNC (22%) and cardiopulmonary (15%). After a mean follow-up of 9.7 +/- 6 months, 16 (39%) patients died. Deceased patients had significantly higher SLEDAI (P = 0.004), and SLICC (P = 0.03) scores. The manifestations associated with mortality were renal disease activity (odds ratio, OR 4.6, confidence interval, CI 95% 1.0-20.6), infections (OR 3.2 CI 95% 2.0-5.3) and thrombocytopenia (OR 4.0, CI 95% 1.0-15.9). The survival at 9.7 months was 72, 62 and 50% in patients with an SLEDAI score of 3-10, 11-20 and > or =21, respectively. The SLEDAI score, the presence of damage and infection were associated with death in patients with acute severe SLE.

Blood leukocyte microarrays to diagnose systemic onset juvenile idiopathic arthritis and follow the response to IL-1 blockade

Systemic onset juvenile idiopathic arthritis (SoJIA) represents up to 20% of juvenile idiopathic arthritis. We recently reported that interleukin (IL) 1 is an important mediator of this disease and that IL-1 blockade induces clinical remission. However, lack of specificity of the initial systemic manifestations leads to delays in diagnosis and initiation of therapy. To develop a specific diagnostic test, we analyzed leukocyte gene expression profiles of 44 pediatric SoJIA patients, 94 pediatric patients with acute viral and bacterial infections, 38 pediatric patients with systemic lupus erythematosus (SLE), 6 patients with PAPA syndrome, and 39 healthy children. Statistical group comparison and class prediction identified genes differentially expressed in SoJIA patients compared with healthy children. These genes, however, were also changed in patients with acute infections and SLE. An analysis of significance across all diagnostic groups identified 88 SoJIA-specific genes, 12 of which accurately classified an independent set of SoJIA patients with systemic disease. Transcripts that changed significantly in patients undergoing IL-1 blockade were also identified. Thus, leukocyte transcriptional signatures can be used to distinguish SoJIA from other febrile illnesses and to assess response to therapy. Availability of early diagnostic markers may allow prompt initiation of therapy and prevention of disabilities.