Session Information
Date: Tuesday, November 7, 2017
Session Title: Systemic Lupus Erythematosus – Clinical Aspects and Treatment Poster III: Therapeutics and Clinical Trial Design
Session Type: ACR Poster Session C
Session Time: 9:00AM-11:00AM
Background/Purpose: Systemic Lupus Erythematosus (SLE) is an autoimmune systemic rheumatic disease that, in our area, presents hematologic manifestations in approximately 70% of cases1. Some of them are very rare so there are no large series whose analysis could provide relevant information._
Objectives: To study the characteristics of patients with Hemophagocytic Syndrome (HS) in a large sample of SLE patients.
Methods: SLE patients from RELESSER database were studied. We analysed the SLE manifestations present at 12 different domains (mucocutaneous, renal, musculoskeletal, constitutional, hematologic, vascular, cardiac, respiratory, neuropsychiatric, gastrointestinal, ophthalmic and serological) before, during and after HS diagnosis and until the last available assessment. We also studied activity (SELENA-SLEDAI) and damage (SLICC/ACR DI) indices in each of those moments.We evaluated the treatment received, HS recurrences and the number of deaths by this entity._
Results: 3,656 patients from 45 Rheumatology Units across Spain were studied. Seven patients with SLE and HS were identified. 71.4% were women, with a mean age (± S.D.) at the diagnosis of SH of 35.1 (± 17.1) years. In 5 of the 7 cases the HS occurred 115.5 (± 162.9) months after the diagnosis of SLE. In the remaining 2 cases the diagnosis of both entities was simultaneous. The main triggers of HS were infections, followed by SLE activity flares. At the time of HS diagnosis, they had high SLE activity with a mean SLEDAI score of 13.1 (± 11.3) and 1.4 (± 2.3) SDI scores._Clinically, 100% of the patients presented fever and alterations of the liver profile, 85.8% cytopenias and 71.5% dermatological manifestations. Respiratory manifestations and hemolytic anemia were present in 57.2% of the cases. Lymph nodes and coagulopathy in 42.9%. Hepatomegaly was detected in 28.6%, as well as neuropsychiatric, digestive and renal manifestations. Splenomegaly was detected in 14.3%. The mean hemoglobin level was 8.6 (± 1.1) g / dl, platelets 85,585 (± 83,390), ferritin 7,410 (± 6,470) ng / ml and triglycerides 404.7 (± 235.6) mg/dl.All patients underwent a bone marrow study._All patients were admitted. They required an average of 2.2 (± 1.5) treatment lines, using 2.8 (± 1.7) drugs. One patient died during the HS episode and another 2 patients had 2 and 3 recurrences respectively.
The following table shows the characteristics of each patient.
|
|
Patient 1 |
Patient 2 |
Patient 3 |
Patient 4 |
Patient 5 |
Patient 6 |
Patient 7 |
|
Number of organ systems affected by SLE before HS diagnosis
|
9 |
4 |
5 |
5 |
6 |
Simultaneous diagnosis of SLE and SH |
Simultaneous diagnosis of SLE and SH |
|
Number of organ systems affected by SLE at HS diagnosis
|
4 |
1 |
2 |
4 |
2 |
4 |
3 |
|
Number of organ systems affected by SLE until last assessment
|
Died |
2 |
2 |
Follow-up lost
|
1 |
5 |
1 |
|
SLEDAI//SLICC-ACR DI at HS diagnosis
|
4//6 |
4//0 |
5//0 |
29//3 |
4//1 |
25//0 |
21//0 |
|
SLEDAI//SLICC-ACR DI 1 year after HS
|
* |
0//0 |
0//0 |
* |
2//1 |
0//0 |
2//13 |
|
SLEDAI//SLICC ACR DI at last assessment
|
* |
0//0 |
0//0 |
* |
0//1 |
2//0 |
2//13 |
|
Number of treatment lines
|
1 |
0 |
3 |
2 |
2 |
4 |
3 |
|
Number
|
2 |
0 |
5 |
3 |
2 |
4 |
4 |
|
Treatments administered
|
GC and CsA
|
GCs, etoposide, iv Ig, CsA, platelets,red cells
|
GC, CYP and iv Ig |
Amphotericin B, miltefosine
|
GC, iv Ig, CsA, MM
|
GC, Cs A, anakinra and CYP
|
|
|
Relapses
|
0 |
2 |
0 |
3 |
0 |
0 |
0 |
|
Deaths
|
Yes |
No |
No |
No |
No |
No |
No |
|
Follow-up time (months)
|
Died |
45 |
80 |
Follow-up lost
|
Unknown |
26 |
24 |
|
GC:Glucocorticoids, iv Ig: intravenous immunoglobulins, |
|
|
|||||
|
CYP: Cyclophosphamide, CsA:Ciclosporine A, MM: Mycophenolate mofetil
|
|
||||||
Conclusion: HS is a rare life-threatening SLE manifestation (<0.5%). It must be suspected in patients with persistent fever who do not respond to antibiotics, cytopenias and evidence of multiorgan involvement._
References: 1 Pego-Reigosa JM et al. Analysis of disease activity and response to treatment in a large Spanish cohort of s with systemic lupus erythematosus. Lupus 2015;24:720-9.
To cite this abstract in AMA style:
Lois-Iglesias A, de Toro FJ, Zea A, Galindo M, Uriarte E, Rúa-Figueroa I, Pego-Reigosa J. Hemophagocytic Syndrome in Patients from SLE Registry from the Spanish Society of Rheumatology (RELESSER) [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/hemophagocytic-syndrome-in-patients-from-sle-registry-from-the-spanish-society-of-rheumatology-relesser/. Accessed October 19, 2021.« Back to 2017 ACR/ARHP Annual Meeting
ACR Meeting Abstracts - https://acrabstracts.org/abstract/hemophagocytic-syndrome-in-patients-from-sle-registry-from-the-spanish-society-of-rheumatology-relesser/