alexa Electroconvulsive Therapy as a Potential Trigger of Adult Onset Still’s Disease | Open Access Journals
ISSN: 2165-7920
Journal of Clinical Case Reports
Make the best use of Scientific Research and information from our 700+ peer reviewed, Open Access Journals that operates with the help of 50,000+ Editorial Board Members and esteemed reviewers and 1000+ Scientific associations in Medical, Clinical, Pharmaceutical, Engineering, Technology and Management Fields.
Meet Inspiring Speakers and Experts at our 3000+ Global Conferenceseries Events with over 600+ Conferences, 1200+ Symposiums and 1200+ Workshops on
Medical, Pharma, Engineering, Science, Technology and Business

Electroconvulsive Therapy as a Potential Trigger of Adult Onset Still’s Disease

Rauber C1*, Ruping FM2, Blank N3 and Merle U1

1Department of Gastroenterology and Hepatology, Division of Rheumatology, University Hospital Heidelberg, Heidelberg, Germany

2Division of Paediatric Surgery, Department of General, Visceral and Transplantation Surgery, University Hospital Heidelberg, Germany

3Department of Hematology, Oncology and Rheumatology, Internal Medicine V, University Hospital of Heidelberg, Germany

*Corresponding Author:
Conrad Rauber
Department of Gastroenterology and Hepatology
Division of Rheumatology, University Hospital Heidelberg
Heidelberg, Germany
Tel: +49 6221 560
E-mail: conrad.rauber@gmx.de

Received Date: April 24, 2017; Accepted Date: May 15, 2017; Published Date: May 20, 2017

Citation: Rauber C, Ruping FM, Blank N, Merle U (2017) Electroconvulsive Therapy as a Potential Trigger of Adult Onset Still’s Disease. J Clin Case Rep 7:958. doi: 10.4172/2165-7920.1000958

Copyright: © 2017 Rauber C, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

Visit for more related articles at Journal of Clinical Case Reports

Abstract

Introduction: Adult onset Still’s disease (AOSD) is an inflammatory disorder of unknown etiology. Macrophage activation syndrome (MAS) is a complication of AOSD and may present with multiorgan failure including cerebral involvement.

Clinical presentation: We describe a 43-year-old female patient with major depression treated with electroconvulsive therapy (ECT). She presented with febrile pharyngitis, arthralgia, rashes, lymphadenopathy, hepatosplenomegaly, liver failure and aseptic meningitis. Elevated serum ferritin and negative blood cultures were consistent with the diagnosis of AOSD complicated by MAS. The patient was treated with corticosteroids and anakinra and recovered rapidly.

Conclusion: The case highlights aseptic meningitis, depression and multiorgan failure as a feature of AOSD/ MAS possibly triggered by ECT. Anakinra is an effective treatment in severe MAS.

Keywords

Adult’s onset still disease; AOSD; Aseptic meningitis; Electroconvulsive therapy; Multiorgan failure; Septicemia

Introduction

Adult’s still disease (AOSD) is an autoinflammatory disease presenting mostly in young adults. Yamaguchi’s criteria has been proposed as a diagnostic criteria. Major criteria are fever, arthralgia, skin rash and elevated neutrophils, minor criteria are sore throat, lymphadenopathy, hepato/-splenomegaly, abnormal liver function and negative tests for antinuclear antibodies (ANA) and rheumatoid factor (RF). Several other features such as pleuritis, pericarditis, abdominal pain and liver failure have also been described [1]. Bacterial sepsis is an important differential diagnosis in patients with a suspected AOSD and organ failure. Serum ferritin has been proposed as a marker to differentiate between septicemia and AOSD [2].

Macrophage activation syndrome (MAS) can be a complication in patients with AOSD which is a potentially life threatening condition. MAS is characterized with hyperactivated mononuclear cells and hyper-phagocytosis of blood cells which leads to severe pancytopenia. Hyperphagocytosis and MAS can be confirmed with a bone marrow aspiration biopsy. However, since phagocytosis cannot be confirmed in every bone marrow biopsy, MAS remains a clinical diagnosis in the majority of patients [3].

Cerebral involvement is a rare complication of AOSD/MAS with unknown etiology [4]. Electroconvulsive therapy (ECT) may be a trigger for cerebral involvement in AOSD/MAS.

Case Report

A 43-year-old female patient presented at the emergency department of a tertiary care center. She had suffered from severe episodes of major depressive disorder (MDD) for nearly two decades. Until six weeks before presentation, she had been treated with 12 cycles of electroconvulsive therapy (ECT) for MDD and concomitant venlafaxin, lamotrigine and valproate.

ECT led to significant improvement in symptoms of depression. Two weeks after her last ECT, she developed gradually worsening new onset arthralgia of her knees. Her general practitioner (GP) prescribed insoles without improvement of the arthralgia.

Seven days prior to admission, she developed a sore throat and spiking temperatures up to 40°C. Her knee arthralgia had worsened significantly, for which her GP prescribed doxycycline which neither relieved the fever nor the pharyngitis. She subsequently developed a maculo-papular rash on her body trunk (Figure 2) which had been considered as an allergic reaction secondary to doxycycline and prednisolone, 1 mg/kg, had been administered. However, the clinical course deteriorated rapidly in the following days, with worsening of her rashes, dysphagia and dyspnea and she was admitted to hospital.

clinical-case-reports-sulci-indicating

Figure 1: FLAIR hyperintensity at the sulci indicating meningitis.

clinical-case-reports-Maculopapular-rash

Figure 2: Maculopapular rash on patient’s back.

Physical examination on admission revealed dyspnea with fine crackles in both lungs, tachycardia, a maculopapular rash on the body trunk, palpable cervical lymph nodes and an acute pharyngitis of the oropharynx. Her body temperature was 41.0°C.

Bacterial septicemia of pulmonary origin was suspected and the patient was started on meropenem and clarithromycin. A whole-body CT scan on the second day after admission revealed low volume ascites and bilateral pleural effusions. Echocardiography showed a moderate pericardial effusion.

Initial laboratory studies revealed an elevated white blood cell count (WBC) of 15,700/μl, lymphocyte count of 500/μl, hemoglobin level (Hb) of 13.4 g/dl and a platelet count (PLT) of 359,000/μl. Lactate dehydrogenase levels were 1788 U/l, total bilirubin 2.6 mg/l, international normalized ratio (INR) 1.51, C-reactive protein was 157.3 mg/l and procalcitonin was 1.27 mg/dl (Table 1). Extensive bacteriology cultures and virology all proved negative. Anti -nuclear antibodies (ANA) and anti-neutrophil-cytoplasmic antibodies (ANCA) were negative and complement C3 and C4 were low to normal. In the clinical course liver and kidney values deteriorated. Aspartate aminotransferase (ASAT) and alanine aminotransferase (ALAT) peaked at 2749 U/l and 9191 U/l respectively 4 days after admission. Liver synthesis and excretion deteriorated subsequently.

Variables   Normal range
White blood cell count (WBC) 15,700/µl 4,000-10,000/µl
Lymphocyte count (LC) 500/µl  
Hemoglobin level (Hb) 13.4g/dl 12-15g/dl
Platelet count (PLT) 359,000/µl 150,000-440,000/µl
Lactate dehydrogenase (LDH) 1788U/l <308U/l
Total bilirubin 2.6mg/dl <1.0mg/dl
International normalized ratio (INR) 1.51 <1.2
C-reactive protein (CRP) 157.3mg/l <5mg/l
Procalcitonin (PCT) 1.27ng/ml <0.05ng/ml
Aspartate aminotransferase (ASAT) 162U/l <50U/l
Alanine aminotransferase (ALAT) 295U/l <50U/l
Gamma-glutamyl transpeptidase(gGT) 520U/l <35U/l

Table 1: Laboratory values on day of admission.

Four days after admission, the patient developed confusion and respiratory distress and was endotracheally intubated for respiratory failure and hypercapnia. She had repeated generalized seizures which responded well to clonazepam. She was started on levetiracetam maintenance therapy.

We performed a cranial magnetic resonance tomography which showed T2 FLAIR hyper-intensive signals at the sulcus (Figure 1). For suspected encephalo-meningitis, acyclovir was added to therapy.

A lumbar puncture revealed an elevated cell count 106/μl (60% lymphocytes, 30% neutrophils) and elevated total protein (0.94 g/dl) in cerebrospinal fluid (CSF). CSF microbial and virological cultures were negative, and findings were consistent with aseptic meningitis. Acyclovir was discontinued.

Serum ferritin on day two after admission was severely elevated with 93,396 μg/l (<300 μg/l). At this point the patient fulfilled 8 out of 8 of Yamaguchi’s criteria. The diagnosis of AOSD and MAS was confirmed and she treated with prednisone, 100 mg, and anakinra, 100 mg per day.

After treatment initiation, the patient recovered rapidly. Kidney and liver function returned to her baseline values and seizures no longer occurred. Her ferritin serum level quickly returned to baseline (Figures 2 and 3). She was extubated after four days of mechanical ventilation.

clinical-case-reports-Serum-ferritin

Figure 3: Serum ferritin level after treatment initiation.

She was discharged from hospital day 14 after treatment began. Anakinra was tapered on outpatient service to 100 mg subcutaneously every second day and prednisone was discontinued. She remains recurrence free 12 months after initial presentation.

Discussion

AOSD is an autoinflammatory syndrome which can present with multiorgan failure and sterile meningitis. We describe a patient with MDD that was refractory to oral medication and ECT was initiated. Two months later the patient developed AOSD, MAS, multiorgan failure and sterile meningitis with seizures. At presentation, she fulfilled Yamaguchi’s criteria for AOSD and recently proposed criteria for MAS in juvenile rheumatoid arthritis [5,6]. CNS involvement is a rare complication and has been estimated to occur in 7% of patients in an early case series on AOSD [7]. There are several case reports with AOSD patients presenting with concomitant aseptic meningitis [4,8]. The etiology of AOSD/MAS remains unclear. Viral infections are discussed as potential triggers in genetically predisposed patients [9,10]. In our patient, arthralgia began shortly after ECT. Single and repeat ECT has been shown to induce a transient immune response due to microglial activation leading to an increase of granulocytes, natural killer cells and monocytes in peripheral blood. 10 ECT also leads to an increase of interleukin-6 (IL-6), interleukin-1β (IL-1β) and tumor necrosis factor α (TNFα) in peripheral blood [11,12]. In comparison to proinflammatory cytokine blood levels in infectious or autoimmune disorders, the magnitude of elevation after ECT is small.

In contrast, in AOSD, these cytokines are largely elevated in peripheral blood. In particular, IL-1β has been implicated in AOSD etiology [13]. IL-1 antagonists (Anakinra) induced more beneficial responses than disease modifying anti-rheumatic drugs (DMARD) in patients with AOSD [14]. In case series of AOSD complicated by MAS, anakinra leads to complete recovery in most patients [15].

In this case report, we hypothesize that the proinflammatory response to ECT may have triggered AOSD with meningeal involvement in our patient. We performed a comprehensive literature review. Until now, there is no published association between ECT and autoimmune disorders. ECT has been successfully used for the treatment of neuropsychiatric lupus erythematosus and autoimmune encephalitis but literature is limited to case reports and focuses on the psychiatric outcome [16,17]. The role of the immunomodulatory effects of ECT in these inflammatory disorders remains unclear. Ultimately, we cannot exclude a mere coincidence of ECT, meningitis and AOSD. It is possible that an unknown viral pathogen triggered AOSD and meningitis.

Conclusion

This is the first case report to describe a potential link between AOSD and ECT. AOSD can mimic severe septic shock and multiorgan failure with cerebral involvement. Treatment with anakinra leads to a rapid and sustained recovery.

References

Select your language of interest to view the total content in your interested language
Post your comment

Share This Article

Relevant Topics

Recommended Conferences

  • Global Experts meeting on Oncology Case Reports
    Aug 29-31, 2017 London, UK
  • Global Experts Meeting on Case Reports
    Osaka, Japan October 09-11, 2017
  • 6th Global Experts Meeting on Medical Case Reports
    October 16-18, 2017 San Francisco, California, USA

Article Usage

  • Total views: 118
  • [From(publication date):
    May-2017 - Jun 23, 2017]
  • Breakdown by view type
  • HTML page views : 99
  • PDF downloads :19
 
 

Post your comment

captcha   Reload  Can't read the image? click here to refresh

Peer Reviewed Journals
 
Make the best use of Scientific Research and information from our 700 + peer reviewed, Open Access Journals
International Conferences 2017-18
 
Meet Inspiring Speakers and Experts at our 3000+ Global Annual Meetings

Contact Us

Agri, Food, Aqua and Veterinary Science Journals

Dr. Krish

agrifoodaquavet@omicsonline.com

1-702-714-7001 Extn: 9040

Clinical and Biochemistry Journals

Datta A

clinical_biochem@omicsonline.com

1-702-714-7001Extn: 9037

Business & Management Journals

Ronald

business@omicsonline.com

1-702-714-7001Extn: 9042

Chemical Engineering and Chemistry Journals

Gabriel Shaw

chemicaleng_chemistry@omicsonline.com

1-702-714-7001 Extn: 9040

Earth & Environmental Sciences

Katie Wilson

environmentalsci@omicsonline.com

1-702-714-7001Extn: 9042

Engineering Journals

James Franklin

engineering@omicsonline.com

1-702-714-7001Extn: 9042

General Science and Health care Journals

Andrea Jason

generalsci_healthcare@omicsonline.com

1-702-714-7001Extn: 9043

Genetics and Molecular Biology Journals

Anna Melissa

genetics_molbio@omicsonline.com

1-702-714-7001 Extn: 9006

Immunology & Microbiology Journals

David Gorantl

immuno_microbio@omicsonline.com

1-702-714-7001Extn: 9014

Informatics Journals

Stephanie Skinner

omics@omicsonline.com

1-702-714-7001Extn: 9039

Material Sciences Journals

Rachle Green

materialsci@omicsonline.com

1-702-714-7001Extn: 9039

Mathematics and Physics Journals

Jim Willison

mathematics_physics@omicsonline.com

1-702-714-7001 Extn: 9042

Medical Journals

Nimmi Anna

medical@omicsonline.com

1-702-714-7001 Extn: 9038

Neuroscience & Psychology Journals

Nathan T

neuro_psychology@omicsonline.com

1-702-714-7001Extn: 9041

Pharmaceutical Sciences Journals

John Behannon

pharma@omicsonline.com

1-702-714-7001Extn: 9007

Social & Political Science Journals

Steve Harry

social_politicalsci@omicsonline.com

1-702-714-7001 Extn: 9042

 
© 2008-2017 OMICS International - Open Access Publisher. Best viewed in Mozilla Firefox | Google Chrome | Above IE 7.0 version