Adult-onset Still's Disease Research Articles

Rare Manifestation of a Rare Disease, Acute Liver Failure in Adult Onset Still's Disease: Dramatic Response to Methylprednisolone Pulse Therapy-A Case Report and Review.

Adult onset Still's disease (AOSD) is a rare systemic inflammatory disorder of unknown etiology. It is characterized by daily fevers, arthralgias or arthritis, typical skin rash, and leukocytosis. Hepatic involvement is frequently observed in the course of AOSD with mildly elevated transaminases and/or hepatomegaly. Fulminant hepatic failure, occasionally requiring urgent liver transplantation, is a rare manifestation of AOSD. Here, we present a case of 22-year-old woman with no significant medical history who initially came with fever, arthralgias, myalgias, generalized weakness, and sore throat. Laboratory data showed mildly elevated transaminases and markedly elevated ferritin levels. She was diagnosed with AOSD based on Yamaguchi diagnostic criteria and was started on prednisone. Three months later, while she was on tapering dose of steroid, she presented with fever, abdominal pain, jaundice, and markedly elevated transaminases. Extensive workup excluded all potential causes of liver failure. She was diagnosed with AOSD associated acute liver failure (ALF). Intravenous (IV) methylprednisolone pulse therapy was started, with dramatic improvement in liver function. Our case demonstrated that ALF can present as a complication of AOSD and IV mega dose pulse methylprednisolone therapy can be employed as a first-line treatment in AOSD associated ALF with favorable outcome.

Successful treatment with tacrolimus of refractory adult-onset Still's disease

Adult-onset Still`s disease (AOSD) is a systemic inflammatory disorder characterized by high fever, arthralgia, transient rash, hepatosplenomegaly, lymphadenopathy, liver dysfunction and leukocytosis. The diagnosis of AOSD is conferred based on clinical findings after elimination of other causes. The mainstay of treatment is glucocorticoids and/or nonsteroidal anti-inf lammatory drugs, although most cases are refractory to these conventional therapies. To treat steroid- resistant AOSD, previous reports have suggested the use of immunosuppressants such as methotrexate (MTX), cyclosporine A (Cy-A), tumor necrosis factor-alpha (TNF-α) blockers, an interleukin (IL)-1 blocker and an IL-6 receptor blocker. Recent studies suggested that higher levels of IL- 18 are detected in patients with active AOSD, which correlate with disease activity and inf lammatory features. In addition, AOSD with high levels of IL-18 is refractory to treatment and is often steroid-resistant. A successful outcome was reported recently in one case of refractory AOSD treated with tacrolimus (Tac), a calcineurin inhibitor similar to Cy-A [1]. Here, we report a case of AOSD with high IL-18 levels that was treated unsuccessfully with steroid in combination with Cy-A, but was subsequently treated successfully using steroid in combination with Tac.

A Case of Adult Onset Still's Disease with Severe Pneumonitis Treated with Anakinra

Adult onset Still’s disease (AOSD) is a systemic autoinflammatory disorder that presents with recurrent fever, extreme fatigue, and joint pain. Pulmonary involvement is not uncommon and, although rare, severe pneumonitis can progress to respiratory failure. Still’s disease-associated pneumonitis is generally treated with immunosuppressive agents, but improvement in our understanding of systemic inflammatory processes led us to explore alternative agents. Anakinra is an interleukin-1 receptor antagonist used to treat autoinflammatory disorders resistant to immunosuppressive therapy. Several case reports have demonstrated efficacy of anakinra in treating AOSD, but its relevance in cases complicated with severe pneumonitis has not been examined. Our patient’s disease activity was not controlled with systemic steroids and cyclophosphamide. Treatment with anakinra led to a dramatic clinical response. This is the first reported case of AOSD with severe pneumonitis refractory to conventional therapy successfully treated with anakinra. (Korean J Med 2014;87:245-250)

Multiple Arthralgia After Use of Amoxicillin in EBV Infection: Could it be Adult Onset of Still’s Disease?

This case discusses about a rare development of adult-onset of still’s disease in a 39-year-old male patient after taking amoxicillin for 14 days in an upper respiratory infection setting. It was confirmed later that he was in fact suffering from EBV infection at that time. Adult Onset Still’s Disease (AOSD) is a rheumatic disease that tends to affect young adults between the ages of 16 and 35. This article presents an uncommon case of AOSD after use of amoxillcin with EBV infection. Still’s disease is thought to be triggered by external infectious agents in genetically predisposed hosts, with many clinical manifestations of AOSD similar to those of self-limited viral infections. Major clinical manifestations include transient fever, rash, arthralgia and arthiritis, myalgia and liver abnormalities. Diagnosis of AOSD is typically based on clinical observation. Treatment for AOSD is empirical and centers on the use of NSAIDs, steroids and disease modifying anti-rheumatic drugs. J Med Cases. 2014;5(2):105-107 doi: https://doi.org/10.14740/jmc1661w

A Case of Musculoskeletal Tuberculosis Mimicking Adult-Onset Still's Disease

A 46-year-old man was admitted to our hospital because of a fever of unknown origin. The patient had a skin rash, arthralgia, and hepatosplenomegaly. Laboratory tests showed leukemoid leukocytosis, a high serum ferritin level (31,200 ng/mL), and abnormal liver function tests. He was initially diagnosed with adult-onset Still’s disease (AOSD). However, imaging studies incidentally detected a cold abscess involving the 11th thoracic vertebra. A biopsy of the abscess revealed histologically granulomatous caseous necrosis, positive for M. tuberculosis by polymerase chain reaction. We present a patient with musculoskeletal tuberculosis (TB) that clinically mimicked AOSD. In TB-endemic countries, extrapulmonary TB should be included in the differential diagnosis of AOSD. (Korean J Med 2014;86:785-790)

Hepatitis E Virus Cross-Reactivity and False-Seropositivity: Challenges to Diagnosis

Hepatitis E Virus (HEV) infection is generally manifested by a self-limiting acute hepatitis E and in some cases, by fulminant hepatic failure [1]. Hepatitis E may be symptomatic or asymptomatic with an overall fatality rate of about 2% worldwide [2]. HEV is an emerging pathogen that has recently evolved to cause chronic liver infections as well as neurologic signs and symptoms [3,4]. Recently, an interesting case of acute hepatitis E, concomitant with rash and arthralgia has been reported, too [5]. Post-viral rashes, arthralgias or Adult-Onset Still’s Disease (AOSD) are known to be triggered by many herpes viruses, adenoviruses, enteroviruses, retroviruses, paramyxoviruses, including rubella and hepatitis viruses (HAV, HBV and HCV) [6]. Previously, an association of acute polyarthritis with hepatitis E has shown evidence for past immunization against herpes viruses, like Cytomegalovirus (CMV) and Epstein-Barr virus (EBV)

Adult Onset Still’s Disease Presenting with Acute Respiratory Distress Syndrome: Case Report and Review of the Literature

Introduction: Adult-onset Still’s disease (AOSD) is a systemic inflammatory disorder characterized by rash, leukocytosis, fevers, and arthralgias. Pulmonary involvement has been reported rarely in AOSD, but acute respiratory distress syndrome (ARDS) is extremely rare and potentially fatal and must be recognized as potential manifestation of underlying AOSD.Methods: We present a case of AOSD manifested by ARDS and review the previously reported cases in Medline/Pub med.Results: Including this case, 19 cases of AOSD complicated with ARDS have been reported in the literature.Conclusions: It is important to recognize ARDS as a manifestation of AOSD so that proper diagnostic and therapeutic management can be initiated promptly.

Adult Onset Still's Disease: A Case Report

Adult onset Still's disease (AOSD) is a chronic multi-system inflammatory disorder characterized by high spiking fever, polyarthralgia and skin rash. Lymphadenopathy is another prominent feature of adult onset Still's disease. We describe a 22 years old lady presented with fever, skin rashes and polyarthritis for 3 months. Examination revealed fever, typical skin rash, generalized lymphadenopathy and polyarthritis. On investigation there were neutrophilic leukocytosis, high ESR, high ferritin level, but RA test and ANA test were negative. All of her history, clinical examinations and laboratory findings fulfill the diagnostic Yamaguchi criteria for AOSD. With proper treatment, now she is completely symptoms free and leaving a healthy life. Anwer Khan Modern Medical College Journal Vol. 4, No. 2: July 2013, Pages 52-54 DOI: http://dx.doi.org/10.3329/akmmcj.v4i2.16944

Reactive haemophagocytic syndrome in a patient with adult-onset Still’s disease: beta-lactams as trigger?

Reactive haemophagocytic syndrome (RHS) is a severe and acquired form of haemophagocytic lymphohistiocytosis that may overlap with adult-onset Still’s disease (AOSD) [1]. The diagnosis of RHS, particularly in its early phase, may be confused with other clinical conditions, such as sepsis, malignancy and rheumatological flare. In November 2012, a 27-year-old woman presented to our hospital with a 3-day history of alternating fever and low back pain. Neurological examination was normal. Tenderness of the lumbar spine on percussion was reported. Blood tests showed a white cell count of 24,130/lL with a normal C-reactive protein (CRP \1.00 mg/L) and erythrocyte sedimentation rate (ESR; 10 mm/h). Hepatic and renal function were normal. Examination of the blood smear did not reveal blasts, and the platelet count and coagulation indices were normal. Urinalysis showed no abnormalities. Procalcitonin (PCT) was 5.7 ng/mL. Fever persisted ([39 C) for 2 days following presentation, and the patient developed signs of haemodynamic instability. Empirical oxacillin was begun awaiting the results of the blood cultures. Emergency imaging scans were performed. A symmetrical oligoarthritis and an intermittent macular–papular rash that extended to the trunk and extremities were detected (Fig. 1). Concomitantly, the levels of CRP (247.6 mg/L), ferritin (up to 15,000 lg/L), lactate dehydrognase (LDH; 2,670 U/L), triglycerides (403 mg/dL), aspartate transaminase (AST; 474 U/L), alanine transaminase (ALT; 171 U/L) started to increase, as did the prothrombin time (12.7 s) and activated partial thromboplastin time (32.4 s). Pancytopenia and reduced fibrinogen levels were also detected. A magnetic resonance imaging (MRI) scan of the spine ruled out epidural abscess and vertebral osteomyelitis. Transthoracic echocardiography did not reveal any valvular vegetations. Positron emission tomography–computed tomography imaging showed no abscess or solid malignancy. Blood and urine cultures grew no bacterial and mycobacterial pathogens. Autoimmunity screening was negative, and radiographs of all involved joints did not reveal marginal erosions. At this point a presumptive diagnosis of AOSD (5/8 criteria) [1] with RHS (4/8 criteria) [1] was strongly considered, and we ruled out infective triggers. Examination of the bone marrow confirmed haemophagocytosis. After the suspension of betalactam treatment, PCT (and other blood tests) progressively normalized. A literature search revealed 34 reported cases of RHS in AOSD in which an active infection was clearly not identified as a potential trigger [1–7]. The authors of these studies proposed that anti-tuberculosis, nonsteroidal antiinflammatory drugs (NSAIDS), sulfasalazine, hydroxychloroquine, gold salts, etanercept and adalimumab, respectively, could have elicited the RHS. In our patient, oxacillin was suspected of acting as the RHS trigger. She received penicillinase-resistant penicillin as a unique medication; moreover, the ‘‘switch-off’’ began just with its suspension. Takeshita [8] and Motegi [9] are the only two authors who advocated treatment with beta-lactams (penicillin G) as the triggering factor for RHS, but in these two F. Mearelli, P. Vinci and M. Gigli were the clinicians involved in the management of the patient.

Clinical value of 18F-fluoro-dexoxyglucose positron emission tomography/computed tomography in patients with adult-onset Still's disease: A seven-case series and review of the literature

Objectives. While there are a few reports describing 18F-fluoro-dexoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) findings in patients with adult-onset Still's disease (AOSD), no summary report has yet been published. In this study, we evaluated the usefulness of FDG-PET/CT for diagnosis and activity evaluation in patients with AOSD by summarizing the findings of our patients and those reported in the literature.Methods. Seven consecutive AOSD patients who had undergone PET/CT at our department between 2007 and 2012 were included. We evaluated FDG uptake for characteristic findings in patients with AOSD. In addition, we reviewed the literature on seven previously reported AOSD patients who had undergone PET/CT.Results. FDG accumulation was positive mainly in the bone marrow (100%), spleen (90.9%), lymph nodes (80.0%) and joints (75.0%). In addition, FDG uptake was positive in the pericardium, pleura, salivary glands, eyelids, muscle and major blood vessels. Six patients underwent follow-up FDG PET/CT for evaluation of treatment efficacy. Follow-up PET/CT showed diminished FDG accumulation in the bone marrow, spleen and lymph nodes, with maximum standardized uptake value (SUVmax) being substantially reduced from 4.03 ± 0.95 to 2.20 ± 0.75 (p = 0.04), 4.04 ± 1.10 to 2.55 ± 1.13 (p = 0.04) and 5.63 ± 4.99 to 2.10 ± 1.91 (p = 0.11), respectively. No significant correlation was found between SUVmax in each lesion and the laboratory data, except for a significant correlation between lactate dehydrogenase (LDH) and spleen SUV.Conclusions. FDG-PET/CT is useful for long-term assessments of AOSD activity in individual patients. However, PET/CT findings alone are not sufficient to make a differential diagnosis of AOSD versus malignant lymphoma.

OR14-002 - ANTI IL-1 therapies and pregnancy outcome

Young women with autoinflammatory diseases on long term IL-1 blockade are increasingly asking about the feasibility, safety and outcomes of pregnancy but few data are available. The FDA classes anakinra as pregnancy risk grade B and rilonacept and canakinumab as grade C. The manufacturers advise that there are no data on the outcome of pregnancy or on excretion into breast milk and that anakinra and canakinumab should only be taken if the benefits outweigh risk; for rilonacept the advice is to avoid in pregnancy. The literature contains only 3 reported cases of use of anakinra in pregnancy – all in adult onset Stills disease (AOSD) and with successful outcomes.

Interleukin-18 as an efficient marker for remission and follow-up in patients with inactive adult-onset Still’s disease

Objectives: Diagnosis of adult-onset Still’s disease (AOSD) is difficult because of a lack of pathognomonic findings and markers. The aim of this study was to investigate the efficacy of interleukin (IL)-18 and free IL-18 in the diagnosis and follow-up of patients with AOSD.Method: Levels of inflammatory cytokines, IL-18, IL-18 binding protein (IL-18BP), and free IL-18 were compared in 80 AOSD patients and 90 controls. The AOSD patients were divided into active and inactive groups according to disease activity, and the inactive patients were subdivided into a remission subgroup and a low disease activity subgroup. We compared erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), ferritin, IL-18, and free IL-18 as disease activity markers in the AOSD patients. Serial serum levels of activity markers were measured in 52 of the 80 AOSD patients at 3- to 6-month intervals.Results:There were significantly higher levels of IL-18 and free IL-18 in the AOSD patients than in the controls. IL-18 and free IL-18 were significantly higher in the active group than the inactive group (p < 0.001 for all). Unlike other activity markers, IL-18 and free IL-18 levels in the low disease activity subgroup were significantly higher than those in the remission subgroup within the inactive group (p = 0.004 and 0.005, respectively). During serial follow-up, ferritin and IL-18 showed a significant decrease in the responder and remission subgroup.Conclusions: IL-18 might be an efficient marker for diagnosis and follow-up of AOSD and might also be a useful predictor of remission, especially in clinically inactive patients.

A case of bilateral trochleitis in adult-onset Still's disease

ObjectivesWe describe the first case of adult-onset Still's disease (AOSD) presenting with trochleitis and successfully treated with an interleukin-1 receptor antagonist. MethodsThis is a descriptive case report of one patient, identified in consultation with the inpatient ophthalmology consult service. ResultsA 23-year-old Caucasian male initially presented with complaints of sore throat, myalgias, rash, and fever. After he failed to respond to antibiotic therapy for presumed streptococcal pharyngitis and developed left eye pain with upgaze, he was admitted to the hospital. Laboratory workup revealed elevated inflammatory markers and liver enzymes. He was eventually diagnosed with AOSD. During his hospitalization he developed binocular vertical diplopia, worsening of left eye pain on upgaze, and exquisite tenderness over the region of the left trochlea, consistent with trochleitis. This diagnosis was supported by magnetic resonance imaging (MRI). Soon after onset of left eye symptoms, the right eye developed identical symptoms. He was started on intravenous corticosteroids, which led to the rapid resolution of his ocular and systemic symptoms. However, after transition to oral corticosteroids, his symptoms recurred. He was started on anakinra, an interleukin-1 (IL-1) receptor antagonist, which led to resolution of his ocular and systemic symptoms. ConclusionsTrochleitis is an unusual form of orbital inflammation localized to the superior oblique tendon and trochlea complex. It has been associated with a number of systemic inflammatory conditions including systemic lupus erythematous, rheumatoid arthritis, and juvenile idiopathic arthritis. This is the first case of a patient with AOSD presenting with trochleitis. Treatment with IV corticosteroids and anakinra resulted in rapid resolution of his ocular and systemic symptoms.

Pseudomaladie de Still et néoplasie : une observation de mélanome et revue de la littérature

IntroductionA malignancy must be carefully excluded before ruling in the diagnosis of adult onset Still's disease (AOSD). However, an occult or poorly symptomatic malignancy can easily be overlooked. Case reportWe report a 50-year-old female patient who presented with features of adult onset Still's disease (AOSD), in fact heralding a malignant melanoma with fatal outcome since discovered lately, at a metastatic stage. In retrospect, the only significant atypical feature was cholestatic hepatitis, which soon disappeared upon institution of glucocorticoid treatment. The literature review identified 27 additional cases of AOSD-like disease associated with malignancy published since 1980 including solid cancer in 61% of the cases (especially breast and lung) and haematological malignancies in 39% of the cases (especially malignant lymphoma). The interval between OASD-like symptoms and malignancy averaged 8 months, and AOSD most often preceding malignancy. Although idiopathic AOSD and neoplastic AOSD-like disease are often indistinguishable initially, some features could point toward the latter: an onset of AOSD after the age of 40 years, the presence of atypical clinical, biological, or immunological features in less than one third of the cases, and a poor response to NAIDS or systemic glucocorticoids in 61% of the cases. ConclusionMaking the differential diagnosis of malignancy-associated AOSD in a timely fashion remains a primary goal, even in the most typical cases and those showing good initial therapeutic response.

Subacute Liver Failure Following Anakinra Treatment for Adult-onset Still Disease

To the Editor: Anakinra is approved for the treatment of rheumatoid arthritis and it is used off-label for various other rheumatic diseases1. Although beneficial effects of anakinra on liver inflammation and regeneration have been shown2, we describe a case of subacute liver failure in a patient with adult-onset Still disease (AOSD) who had been treated with anakinra monotherapy. Three months before admission to our hospital, the 20-year-old man had been diagnosed with AOSD. Initial treatment consisted of anakinra 100 mg and prednisolone 100 mg daily. The latter had been tapered to 10 mg after achieving remission 1 week before the first symptoms of liver failure appeared. The patient developed fever and jaundice. C-reactive protein was increased to 42 mg/dl, immunoglobulin E (IgE) was elevated to 709 kU/l, and transaminases were substantially increased, with alanine transaminase levels of 1128 U/l (Figure 1a). With an INR of 2.7, coagulation was massively disturbed. Figure 1. Development of the clinical course and hematoxylin and eosin stainings of the liver biopsy. (a) Development of alanine transaminase (ALT, U/l), bilirubin … Address correspondence to Dr. Benten; E-mail: d.benten{at}uke.de

Prurigo pigmentosa as an atypical persistent plaque-like skin rash in adult-onset Still’s disease: case report and literature review

Adult-onset Still’s disease (AOSD) is a systemic inflammatory disease of unclear etiology. It is classically characterized by spiking fevers, arthralgias, leukocytosis and a typical rash. Usually most of these clinical findings are not specific to this disease and it is often a diagnosis of exclusion. The exception to this is usually the typical maculopapular nonpruritic salmon-colored eruption, which has been shown to have high sensitivity and specificity for the diagnosis of patients with AOSD. It is important to be aware, however, that some patients also present with an atypical rash. Prurigo pigmentosa is a distinctive inflammatory disease first described in Japan. The patient in our case presentation was diagnosed with AOSD based on Yamaguchi criteria and also had a diffuse pruritic persistent hyperpigmented rash on the back, chest and distal extremities with a biopsy revealing histopathology and clinical findings consistent with the diagnosis of prurigo pigmentosa. This review of literature was carr...

Tocilizumab for the treatment of adult-onset Still’s disease: results from a case series

Adult-onset Still's disease (AOSD) is a chronic inflammatory disease of unknown etiology which commonly affects young adults. Treatment of AOSD patients includes nonsteroidal anti-inflammatory drugs, corticosteroids, and DMARDs. Interleukin (IL)-6 blockade is an attractive therapeutic option for AOSD because this cytokine contributes to the pathogenesis of major AOSD symptoms. Tocilizumab (TCZ) is a humanized anti-IL-6 receptor antibody that blocks the effects of IL-6. Preliminary results of TCZ in AOSD have been promising. Here, we reported our experience evaluating both the safety and the efficacy of 12months therapy with TCZ in 11 patients with AOSD refractory to corticosteroids and MTX therapy, followed for 18months, including the first 12months of active treatment and the last 6months to evaluate the activity of the disease when the treatment was discontinued. The main outcome measures were the European League Against Rheumatism (EULAR) improvement criteria and improvement of systemic symptoms at the 3, 6, 12, and 18-months follow-up periods. Our patients rapidly responded and experienced a sustained clinical remission over time during active treatment. Disease Activity Score 28 decreased from 5.62 (3.75-8.28) [median (range)] at baseline to 1.61(0.49-3.5) at month 12. EULAR remission was achieved in 81.82% at 12months. Tender joint and swollen joint counts displayed a progressive reduction during active therapy study period. During treatment, we observed a resolution of fever in our AOSD patient. In conclusion, TCZ might represent a suitable option for the therapy of refractory AOSD patients.

A Patient with a Phenotype of Adult-onset Still Disease, But a Genotype Typical of Cryopyrin-associated Periodic Fever Syndrome

To the Editor: Cryopyrin-associated periodic fever syndrome (CAPS) is a group of genetic diseases consisting of familial cold autoinflammatory syndrome (FCAS) and presenting with urticaria triggered by cold, Muckle-Wells syndrome (MWS) with fever, hearing loss, rash and joint pain, and neonatal onset multisystem inflammatory disease (NOMID), a severe neonatal disease. CAPS is associated with mutations in the NLRP3 gene1,2. NLRP3 associates with the proteins ASC and pro-caspase 1 to form the NLRP3 inflammasome, which is important for activation of pro–inter-leukin 1β (IL-1β) to mature IL-1β. Consequently, CAPS can be treated with anti-IL-1β therapy. Some patients with more diffuse inflammatory symptoms together with NLRP3 mutations have been classified as “atypical” CAPS3. We describe a patient with inflammatory symptoms fulfilling criteria of adult-onset Still disease (AOSD) but with a genotype typical of CAPS. A 32-year-old man with previously known thrombophlebitis related to activated protein C resistance presented in May 2009 with sore throat, relapsing fever above 39°C especially in the afternoon, cervical adenitis, and transient maculopapular exanthema. C-reactive protein (CRP) was 192 mg/l, leukocyte count 11.3 × 109/l (ref < 8.8), neutrophils 8.2 × 109/l, aspartate aminotransferase 1.6 μkat/l (ref < 0.76), alanine aminotransferase 2.5 μkat/l (ref < 1.2), and lactate dehydrogenase 9.7 μkat/l (ref < 3.5). … Address correspondence to Dr. P. Eriksson, Department of Rheumatology, University Hospital, 581 85 Linköping, Sweden. E-mail: per.eriksson{at}lio.se

Liver transplantation for acute liver failure caused by macrophage activation syndrome

Macrophage activation syndrome (MAS) is a rare, potentially fatal condition, which most frequently complicates rheumatological conditions and is often associated with liver dysfunction. In this case report of a patient with MAS, acute liver failure developed despite conventional immunosuppressive therapy. Liver transplantation resulted in rapid recovery and the patient has remained well for six years. A recent diagnosis of Adult Onset Still's Disease (AOSD) provides additional supporting evidence that the initial presentation was caused by MAS. While transplantation in the context of systemic disease remains controversial, this first reported case of successful adult liver transplantation for acute liver failure caused by MAS raises an interesting clinical dilemma.

Demyelinating encephalopathy in adult onset Still's disease: Case report and review of the literatures

Adult onset Still’s disease (AOSD) is an immunologic disorder haracterized by high spiking fever, arthritis, evanescent rash, lymhadenopathy, myalgia and leukocytosis. Sometimes liver, lung, eart, gastrointestinal tract and central nerve system may be nvolved [1]. AOSD has been recognized as a specific systemic form f juvenile rheumatoid arthritis. Cerebral involvement is not often ound. Symptoms such as high intracranial pressure, sudden onset f unconsciousness, and polyneuropathy have been reported in atients with AOSD [2,3]. Diagnosis is difficult since the clinical eatures are variable. Here we report a case with complication f demyelinating encephalopathy that developed rare neurologial features as headache, slight hemiparalysis and sudden onset of nconsciousness with tumor-like imagings on MRI. However, simlar symptoms and imaging findings in patients with adult onset till’s disease are not found in the literatures.