POSTER VIEWING I
Tuesday 1 May 2018
POSTER VIEWING I
POSTER ABSTRACT SESSION
001 SUCCESSFUL TREATMENT OF EPSTEIN BARR VIRUS-
TRIGGERED MACROPHAGE ACTIVATION SYNDROME IN A
PATIENT WITH SYSTEMIC JUVENILE IDIOPATHIC ARTHRITIS
Owen Cronin1, Euan McRorie1 and Mohini Gray1,2
1 mab (anti-TNF-a) who developed GCA and has subsequently been
Rheumatic Diseases Unit, Western General Hospital, Edinburgh,
UNITED KINGDOM, and 2MRC Centre for Inflammation Research,
The University of Edinburgh, Edinburgh, UNITED KINGDOM
Background: Macrophage activation syndrome (MAS) is a potentially
life-threatening complication of inflammatory rheumatic disorders,
most commonly occurring in patients with systemic-onset juvenile
idiopathic arthritis (JIA). It is characterised by an inappropriate,
aggressive cascade of pro-inflammatory cytokine release from innate
immune cells, driven by dysfunctional activation and uncontrolled
expansion of macrophages and T-lymphocytes. Recognition of the
clinical features of MAS is the first step in preventing mortality.
Mortality rates in patients with JIA are in the region of 8%. Episodes of
MAS triggered by host infection pose challenging therapeutic
dilemmas for the treating clinician, including careful consideration of
the balance between effective pharmacological blockade of immune
over-stimulation, and avoidance of excessive immunosuppression in
the face of pathogenic microbial threat.
Methods: We performed a retrospective case review of the success-
ful management of MAS triggered by significant Epstein Barr virus
(EBV) infection, in a patient with systemic JIA in the rheumatic diseases
unit, Edinburgh.
Results: We report a case of a 22-year-old male with JIA, presenting
with unremitting high fevers, widespread lymphadenopathy and
splenomegaly. Preceding history suggested a viral prodrome prior to
presentation to the emergency department. Laboratory abnormalities
on presentation including thrombocytopenia (platelet count of 69
x109), hyperferritinemia (15,433 mg/l), hypertriglyceridemia (2.1mmol/
L), and low fibrinogen levels (1.2g/L), led to a diagnosis of MAS in
accordance with the 2016 ACR/EULAR classification criteria. A
thorough diagnostic work-up was performed revealing extremely
elevated titres of EBV DNA (titre ¼ 28,746 IU/L) in the peripheral
blood. Clinical concerns of an EBV-associated lymphoma were
eliminated following bone marrow biopsy and axillary lymph node
sampling that revealed the presence of EBV-positive cells but no
evidence of an EBV-associated lymphoproliferative disorder. In this
case, the patient’s usual immunosuppression was withheld on
presentation (weekly oral methotrexate (5mg) and subcutaneous
injections of the humanised anti-IL-6 receptor monoclonal antibody,
tocilizumab). The patient was treated with intravenous methylpredni-
solone (3 pulses of 1000mg) and one dose of intravenous immunoglo-
bulin (2g/kg). He was monitored as an inpatient for six days with
supportive care including IV fluids and analgesia. Over the course of
eight weeks EBV DNA viral load reduced without the need for anti-viral
treatment and our patient made a full recovery. Usual immunosup-
pression was recommenced at five weeks post-presentation.
Conclusion: Epstein Barr virus is an acknowledged trigger for macro-
phage activation syndrome in patients with autoimmune rheumatic
disease. In this case MAS was successfully treated with intravenous
immunoglobulin with concurrent cessation of immunosuppression
following identification of the causative organism. We propose that
treatment of MAS in rheumatic disorders may be stratified based on the
precipitating cause e.g. viral, bacterial infection or others.
Disclosures: The authors have declared no conflicts of interest.
002 BIOPSY PROVEN GIANT CELL ARTERITIS IN A PATIENT
WITH PSORIATIC ARTHRITIS ON TNF-ALPHA INHIBITOR
TREATED WITH HIGH DOSE PREDNISOLONE AND A SWITCH
TO SECUKINUMAB (ANTI IL-17)
Luke Sammut1, Anna Litwic1, Richard Smith1 and Sarah Bartram1
1
Rheumatology, Salisbury NHS Foundation Trust, Salisbury, UNITED
KINGDOM
Background: Corticosteroids remain the mainstay of treatment in
giant cell arteritis (GCA). Corticosteroids treat GCA by selectively
Tuesday 1 May 2018 iii43
suppressing IL-1b, IL-6 and IL-23-releasing antigen-presenting cells,
disrupting induction of Th17 cells. Corticosteroids are however
associated with significant morbidity including osteoporosis, diabetes
mellitus, hypertension and infection. At least two distinct CD4 T-cell
subsets promote vascular inflammation in GCA and as a consequence
a number of cytokines have been implicated in the pathogenesis of the
disease including TNF-a, IL-1 and IL-6, IFN-g and IL-17. Trials looking
at TNF-a inhibitors have been disappointing whilst trials of IL-1, IL-6
and IL-23 blockade are ongoing. In light of this, we report a case of a
patient with a background of psoriatic arthritis treated with adalimu-
managed successfully with the IL-17 inhibitor secukinumab, licensed
for use in psoriatic arthritis, psoriasis and ankylosing spondylitis.
Downloaded from https://academic.oup.com/rheumatology/article/57/suppl_3/key075.225/4971125 by guest on 22 January 2024
Methods: A 70 year old male was admitted with a one week history of
fever, dry cough, and occipital headache. Past medical history
included psoriatic arthritis, diagnosed in 2013, and managed with
fortnightly 40mg subcutaneous injections of adalimumab since
February 2015 with remission achieved shortly after its introduction.
Results: On admission investigations showed a CRP of 233mg/L, a
WBC of 14.2 x109/L. (3.5-11.0) and negative blood cultures. His chest
x-ray, T-spot and sputum culture were normal. He was started on
broad spectrum antibiotics for sepsis of unknown origin but had no
clinical response and the temperature and CRP remained elevated.
Further investigations including a CT scan of his head, chest, abdomen
and pelvis, and lumbar puncture with CSF analysis were carried out
and did not demonstrate any source of sepsis. A left temporal artery
biopsy was performed and the histological features were characteristic
of GCA. He was started on 40mg of oral prednisolone, and his fevers
and inflammatory markers settled within 48 hours. He remained on
adalimumab but his psoriatic arthritis flared and corticosteroid
requirement for the GCA remained high. Three months later adalimu-
mab was stopped and he was started on secukinumab (anti-IL-17). At
review four months later both the psoriatic arthritis and GCA were in
remission and his CRP had normalised on low dose corticosteroids.
Nine months after presentation with GCA, this man remains well on low
dose corticosteroids and secukinumab.
Conclusion: There is evidence to suggest that IL-17 is implicated in
the pathogenesis of GCA along with other cytokines. Whether the
remission is sustained when corticosteroids are stopped remains to be
seen.
Disclosures: The authors have declared no conflicts of interest.
003 CARDIAC SARCOIDOSIS IDENTIFIED ON MRI OR
PET-CT
Ella Daniels1, Lucia Chen1, Catherine Hughes1, Shirish Sangle1 and
David D’Cruz1
1
Rheumatology, Guy’s and St Thomas’ NHS Foundation Trust,
London, UNITED KINGDOM
Background: Sarcoidosis is a multisystemic non-caseating granulo-
matous disorder of unknown aetiology. Clinical manifestations of
cardiac sarcoidosis vary depending on the location and extent of
granulomatous inflammation. It can occur insidiously, present with
conduction abnormalities, heart failure or sudden cardiac death.
Only 5-10% of cases present with serious cardiac dysfunction,
whereas up to 50% of patients can be asymptomatic.
For these reasons cardiac sarcoidosis remains a diagnostic challenge.
Diagnosis relies on Positron Emission Tomography - Computed
Tomography (PET-CT), cardiac Magnetic Resonance Imaging (MRI),
or histopathology. This poster will compare two cases of cardiac
involvement identified on cardiac MRI and PET-CT in patients with
known sarcoidosis.
Methods: This is a case report describing two cases. Full informed
written patient consent was obtained.
Results: Case one is a 48 year old female with pulmonary sarcoidosis,
who presented with a new rash, intermittent pyrexia and a vague
history of unexplained arrhythmias. ACE was elevated at 146. She
underwent a PET-CT scan, which demonstrated focal diffuse uptake
within the myocardium. This was in keeping with active myocarditis
secondary to sarcoidosis. There were no specific abnormalities
identified on her cardiac MRI.
Case two is a 56 year old female with lupus and sarcoid overlap. She
presented with weight loss and breathlessness with a dramatic
troponin and BNP rise. PET-CT findings were non-specific, however
cardiac MRI found focal epicardial fibrosis in the basal anterolateral