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Scientific Abstracts 1497
Table 1. Patient characteristics
Characteristic N=16
Age (years) 26 (19.5 – 30)
Duration of symptoms (days) 60 (18.7 – 90)
Fever 16 (100)
Pancreatitis 1 (6.3)
Renal 7 (43.8)
Neurological 7 (43.8)
Myocarditis 6 (37.5)
Hepatomegaly 7 (43.8)
Splenomegaly 3 (18.8)
Lymphadenopathy 12 (75)
Anaemia 16 (100)
Leukopenia 13 (82)
Thrombocytopenia 14 (88.2)
Coagulopathy 7 (43.8)
Concomitant infection 3 (18.8)
H score 222 (193 – 254)
cSLE-MAS diagnostic criteria 16 (100%)
HLH 2004 (≥5) 7 (43.8)
Corticosteroids 16 (100%)
Cyclophosphamide 8 (50%)
Intravenous immunoglobulin 2 (12.5%)
Cyclosporine 6 (37.5%)
Death 4 (25%)
All data presented as n (%) and median (IQR)
cSLE-MAS – Childhood systemic lupus erythematosus – Macrophage activation syndrome,
HLH – Hemophagocytic lymphohistiocytosis
had been successfully treated, 27.8% had metastasis, 5.6% had died. There
was no significant difference in SLEDAI between patients with cancer and
patients without. Whereas malignancy is correlated to longer disease dura-
tion (p= 0.01) and older age of SLE onset with significant difference (p=
0.001).
Conclusion: Although we have detected an increasing incidence of cancer in
SLE patients in comparison to normal population, our study didn’t nd a sign-
cant correlation between SLE disease activity and the risk of cancer. We should
closely observe SLE patients with old age at onset and/or long disease duration
because of their higher risk for cancer development.
References:
[1] Bernatsky S, Ramsey-Goldman R, Joseph L, et al (2014). Lymphoma risk in
systemic lupus: effects of disease activity versus treatment. Ann Rheum Dis,
73, 138–42.
[2] Hochberg MC (1997). Updating the American College of Rheumatology
revised criteria for the classication of systemic lupus erythematosus. Arthri-
tis Rheum, 40, 1725.
[3] Bombardier C, Gladman DD, Urowitz MB, Caron D, Chang CH (1992) Deri-
vation of the SLEDAI. A disease activity index for lupus patients. The Com-
mittee on Prognosis Studies in SLE. Arthritis Rheum:630–640.
Disclosure of Interests: None declared
DOI: 10.1136/annrheumdis-2020-eular.5044
AB0395 SYSTEMIC LUPUS ASSOCIATED MACROPHAGE
ACTIVATION SYNDROME – A SINGLE CENTRE
EXPERIENCE FROM INDIA
N.Acharya1, S.Jha1, D.Mishra1, A.Chattopadhyay1, S.Jain1, S.
K.Sharma1, A.Sharma1, S.Jain1, V.Dhir1. 1Post Graduate Institute of
Medical Education and Research, Division of Rheumatology, Department of
Internal Medicine, Chandigarh, India
Background: Macrophage activation syndrome (MAS) is a potentially fatal com-
plication of systemic lupus erythematosus (SLE).(1) Overlapping clinical features
of SLE activity with MA makes it a clinical diagnosis. There is limited data from
developing countries like India.
Objectives: To study the clinical features, treatment, and outcomes in
SLE-MAS.
Methods: This single-centre, retrospective study included patients fulfill-
ing SLICC 2011 criteria for SLE, admitted from January 2017 to Novem-
ber 2019, and diagnosed as MAS by a team of attending physicians. Their
demographic and clinical data, treatment, and outcomes were recorded. H
score and SLEDAI were calculated. Patients were assessed for HLH classi-
fication criteria (2004) and preliminary diagnostic criteria for MAS in child-
hood SLE (cSLE).
Results: Sixteen patients (median age – 26 years, 15 females) were
included. Twelve patients (75%) had MAS as the initial presentation of SLE.
The common clinical features were fever (100%) and cytopenias (100%).
The mean duration of symptoms was 60 days. The most frequent bio-
chemical abnormalities were high ferritin (>500 ng/ml, 100%) and elevated
transaminases (100%, aspartate transaminase > alanine transaminase).
Common complications were renal (43.8%), neurological (43.8%), and
coagulopathy (43.8%). Seven and 16 patients fulfilled the HLH 2004 and
cSLE – MAS preliminary criteria, respectively. The median H score was 222,
giving a cumulative probability of 96%. All the patients received high-dose
steroids. Cyclophosphamide pulse and cyclosporine were administered to 8
(50%) and 6 (37.5%) patients respectively. There were four (25%) in-hospital
mortalities.
Table 2. Laboratory features
Parameter N = 16
Haemoglobin (g/dL) 6.9 (5.7 – 7.8)
Total leucocyte count (cells/mm3) 1400 (1025 -3175)
<1000 3 (18.8)
1000 – 2500 9 (56.3)
2500 - 4000 1 (6.3)
Platelet (cells/mm3) 57500 (23500 – 95250)
<20000 3 (18.8)
20000 – 50000 3 (18.8)
50000 – 1 lac 8 (50)
Serum ferritin > 500 ng/ml 16 (100%)
Fibrinogen 2.48 (1.6 – 4)
Triglycerides (mg/dL) 375 (294 -470)
AST/ALT (U/L) 153 (113 – 234) / 90 (68 – 170)
Procalcitonin (pg/mL) 0.8 (0.3 – 1.4)
Lactate dehydrogenase (U/L) 1674 (1081 – 2184)
Erythrocyte sedimentation rate (mm in 1st hour) 61 (44 – 69)
C- reactive protein (mg/L) 36 (6.3 – 52)
Bone marrow examination 14 (87.5)
Increased histiocytes with hemophagocytosis 5 (32.5)
All data presented as n (%) and median (IQR)
ALT – Alanine transaminase, AST – Aspartate transaminase
Conclusion: Fever, cytopenia, high ferritin, and elevation of transaminases were
the commonest features in this series of SLE-MAS. SLE-MAS carried a high
mortality (25%) despite aggressive treatment.
References:
[1] Gavand P-E, Serio I, Arnaud L, Costedoat-Chalumeau N, Carvelli J, Dossier
A, et al. Clinical spectrum and therapeutic management of systemic lupus
erythematosus-associated macrophage activation syndrome: A study of 103
episodes in 89 adult patients. Autoimmun Rev. 2017;16(7):743–9.
Acknowledgments: NONE
Disclosure of Interests: None declared
DOI: 10.1136/annrheumdis-2020-eular.5703
AB0396 DIAGNOSTIC PROTOCOL INCLUDING MINIMALLY
INVASIVE MINOR SALIVARY GLANDS BIOPSY IN
SJÖGREN’S SYNDROME IN A SPANISH REFERENCE
CENTER
J.Álvarez Troncoso1, C. M.Oñoro López1, C.Soto Abánades1, E.Ruiz Bravo2,
L.Ramos Ruperto1, L.Blasco Santana2, R.Sorriguieta Torre1, Á.Robles
Marhuenda1, E.Martínez Robles1, A.Noblejas Mozo1, J. J.Rios1, F.Arnalich
Fernández1. 1Hospital Universitario La Paz, Department of Internal Medicine,
Madrid, Spain; 2Hospital Universitario La Paz, Pathological Anatomy, Madrid,
Spain
Background: Sjögren’s syndrome (SjS) is a systemic autoimmune disease
with a broad clinical presentation from dry syndrome to systemic extraglan-
dular manifestations. The diagnosis can be complex since none of the mark-
ers, except anti-Ro, is sufficiently sensitive or specic. Although, minor salivary
glands biopsy (MSGB), Schirmer’s test and unstimulated whole salivary ows
(UWSF) are the hallmark for the diagnosis of this entity, its use is not wide-
spread in some centers.
Objectives: The aim of the study was to analyze the usefulness and safety of
the diagnostic protocol for the classication of SjS and the immunological and
analytical markers in dry syndrome due to SjS.
on March 21, 2021 by guest. Protected by copyright.http://ard.bmj.com/Ann Rheum Dis: first published as 10.1136/annrheumdis-2020-eular.4921 on 2 June 2020. Downloaded from
1498 Scientific Abstracts
Methods: Prospective observational study of a cohort of patients with
sicca syndrome from a reference center. The diagnostic protocol (Schirm-
er’s test, UWSF and minimally invasive MSGB) was applied in the same
consultation. Demographic, clinical, analytical and histological data were
reviewed.
Results: Over a period of 6 months, 48 patients with dry syndrome were
analyzed, of which 39 women (81.2%). The main suspicion was SjS (39),
followed by sarcoidosis (3), IgG4-related disease (2) and other diagnoses
(4). The mean age was 59.1±4.4 years. Almost half (45.8%) reported xeros-
tomia and 41.6% xerophthalmia. Recurrent parotidomegaly was described in
6 patients (12.5%) and arthralgias in 12 (25%). Immunologically, 23 (47.9%)
presented anti-nuclear antibodies, 13 (27.1%) anti-Ro, 4 (8.3%) anti-La, 12
(25%) rheumatoid factor and 15 (31.2%) low C4. Schirmer test was positive
in 32 patients (66.7%), UWSF in 22 (45.8%) and 9 (18.8%) had a focus score
≥1, although 16 (33.3%) had focal lymphocytic sialadenitis in the MSGB.
A total of 21 (43,8%) patients were classified according to the 2016 ACR/
EULAR criteria. 12 (57.1%) were seropositive SjS and 9 (42.9%) seroneg-
ative SjS. MSGB sensitivity was 71% and specificity 96%. Patient reported
symptoms were unhelpful to differentiate SjS from other causes of dry syn-
drome. The number of protocols needed to diagnose a SjS was 2.28 (5.33
in seronegative SjS). Complications associated with the procedure were low
(1 of 48) and mild (self-limited paraesthesia). Patients with SjS, unlike those
with dry syndrome of another etiology, had more anemia (p<0.001), lym-
phopenia (p=0.022), ESR (p=0.030), beta-2 microglobulin (p=0.011), ANA
(p<0.001), anti-ENA (p=0.006), anti-Ro (p<0.001), low C4 (p<0.001) and
hypergammaglobulinemia (p=0.002).
Conclusion: Immunological and histological manifestations were more predic-
tive than clinical ones to differentiate SjS from other causes of dry syndrome.
MSGB is a simple, sensitive, specic and safe procedure. The application of the
diagnostic protocol (Schirmer test, UWSF and MSGB) allowed to standardize the
classication of SjS and increased the diagnosis of patients with seronegative
SjS.
References:
[1] Ramos-Casals M, Brito-Zerón P, Bombardieri S On behalf of the EULAR-
Sjögren Syndrome Task Force Group, et al. EULAR recommendations for
the management of Sjögren’s syndrome with topical and systemic therapies.
Annals of the Rheumatic Diseases 2020;79:3-18.
[2] Guellec D, Cornec D, Jousse-Joulin S, et al. Diagnostic value of labial minor
salivary gland biopsy for Sjögren’s syndrome: a systematic review. Autoim-
mun Rev. 2013;12(3):416–420.
Disclosure of Interests: None declared
DOI: 10.1136/annrheumdis-2020-eular.4921
AB0397 LUPUS FATIGUE PROBLEMS IN THE RUSSIAN
COHORT OF PATIENTS WITH SYSTEMIC LUPUS
ERYTHEMATOSUS (RENAISSANCE).
E.Aseeva1, S.Solovyev1, G.Koilubaeva2, N.Nikishina1, E.Nasonov1,
A.Lila1. 1V.A. Nasonova Research Institute of Rheumatology, Moscow,
Russian Federation, Intensive Care Department, Moscow, Russian
Federation; 2National Center for Cardiology and Internal Medicine named
after Academician M. Mirrakhimov, Bishkek, Kyrgyz Republic, Rheumatology,
Bishkek, Kyrgyzstan
Background:
Objectives: To determine the dependency of fatigue from SLE activity,
irreversible organ damage and HRQoL in SLE patients of the Renaissance
cohort.
Methods: 328 Russian SLE patients were enrolled in the study (M/F 30/298)
who fullled SLICC 2012 criteria. The SLEDAI 2K activity, SLICC damage index,
Facit Fatigue scale, and health related quality of life (HRQol) using the LupusQol
questionnaire were evaluated.
Results: Based on the Facit Fatigue scale scores fatigue was veried in 148
(45%) out of 328 patients with SLE. Following lupus fatigue status patients were
divided into two groups - 148 and 168 patients respectively. The groups were per-
fectly matched in terms of age, duration of the disease, duration of GCs therapy,
and damage scores (Table1)
The SLEDAI 2K activity scores were signicantly higher in the group with lupus
fatigue - 9.6±6.0 vs 6.7±4.2 (p=0.01) as compared to values in the group without
fatigue, as well as the level of antibodies to DNA (p=0.02), 110.2±34.2 and
82.3±20.5, respectively.
There was a signicant decrease in HRQoL in patients with lupus fatigue based
on scores in all eight scales of the questionnaire (p=0.00),
Figure 1. Comparative assessment of HRQoL using the LupusQol questionnaire in 328 SLE
patients with and without fatigue.
Conclusion: Almost half (45%) SLE patients in the Russian cohort experience
fatigue. It is associated with disease activity on the SLEDAI 2k scale and high
levels of antibodies to DNA. All patients experiencing lupus fatigue have signif-
icantly worse HRQoL based on scores in all eight scales of the questionnaire
LupusQol.
Disclosure of Interests: None declared
DOI: 10.1136/annrheumdis-2020-eular.3167
AB0398 TNF-LIKE WEAK INDUCER OF APOPTOSIS / FGF
INDUCIBLE MOLECULE 14 PATHWAY IN UNTREATED
LUPUS NEPHRITIS: SERUM OR URINE TWEAK
LEVELS MORE ACCURATE IN RELATION TO RENAL
ACTIVITY?
M.Bekhit1, N.Abaza1, N.Kamel1, M.Ossman1, S.Saad El Din2. 1Ain Shams
University, Rheumatology, Cairo, Egypt; 2Ain Shams University, Pathology, Cairo,
Egypt
Background: Lupus nephritis (LN) is one of the most serious manifestations of
SLE, affecting 70% of patients and the most critical predictor of morbidity and
mortality of the disease [1].Tumor necrosis factor-like weak inducer of apopto-
sis/ broblast growth factor inducible molecule 14 (TWEAK/Fn14) activation is
involved in various pathological processes that occur locally in kidneys, facilitat-
ing the pathogenesis of LN [2].
Objectives: To assess serum and urine TWEAK levels as well as renal Fn14
expression in newly diagnosed patients with LN and its correlation to disease
activity.
Methods: The present study included 30 selected newly diagnosed previ-
ously untreated SLE patients divided into 2 groups; 15 patients with LN and
15 without LN as well as 30 age and sex matched healthy subjects who
served as control group. Written consent was obtained from all patients and
Table 1. Comparative analysis of 328 SLE patients with and without
lupus fatigue
Characteristics
mean (SD)
Patients with
lupus fatigue
(n=148)
Patients without
lupus fatigue
(n=168)
P
(Mann-
Whitney)
Age in years 35,31±12,03 33,68±10,68 0,13
Disease durationin in years 9,57±9,47 10,1±9,17 0,6
Duration of glucocorticoid therapy in month 72,81±7158 79,43±75,98 0,6
SLICC DaI, score 1,55±1,37 1,69±1,23 0,44
C3 Mu\ml 0,88±0,33 0,87±0,31 0,75
C4 Mu\ml 0,15±0,12 0,16±0,14 0,36
ANF, hep2 641± 453 550± 402 0,4
Fatigue scores by the Facit fatigue scale 25,16±6,58 42,43±4,56 0,000
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