Hemophagocytic Syndrome in Patients from SLE Registry from the Spanish Society of Rheumatology (RELESSER)

Ana Lois-Iglesias¹, Francisco J. de Toro², Antonio Zea³, María Galindo⁴, Esther Uriarte⁵, Iñigo Rúa-Figueroa⁶ and JM Pego-Reigosa⁷, ¹Rheumatology, University Hospital A Coruña, A Coruña, Spain, ²Rheumatology Division, INIBIC-Complejo Hospitalario Universitario A Coruña (CHUAC), A Coruna, Spain, ³Hospital Ramón y Cajal. Madrid, Madrid, Spain, ⁴Servicio de Reumatología, Hospital 12 de Octubre, Madrid, Spain, ⁵Reumatología, Hospital de Donosti, Donosti, Spain, ⁶Rheumatology Division, Hospital Doctor Negrin, Las Palmas GC, Spain, ⁷Rheumatology Section, Hospital de Meixoeiro, Pontevedra, Spain, Vigo, Spain

Meeting: 2017 ACR/ARHP Annual Meeting

Date of first publication: September 18, 2017

Keywords: Systemic lupus erythematosus (SLE)

Session Information

Date: Tuesday, November 7, 2017

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	0	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: ¹Pego-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.

Disclosure: A. Lois-Iglesias, None; F. J. de Toro, None; A. Zea, None; M. Galindo, None; E. Uriarte, None; I. Rúa-Figueroa, None; J. Pego-Reigosa, None.

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 .

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