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 Table of Contents  
Year : 2021  |  Volume : 16  |  Issue : 1  |  Page : 115-116

Macrophage activation syndrome as the presenting manifestation of systemic lupus erythematosus

Department of Clinical Immunology and Rheumatology, King George's Medical University, Lucknow, Uttar Pradesh, India

Date of Submission29-Aug-2020
Date of Acceptance06-Oct-2020
Date of Web Publication23-Mar-2021

Correspondence Address:
Prof. Anupam Wakhlu
Department of Clinical Immunology and Rheumatology, King George's Medical University, Lucknow - 226 003, Uttar Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/injr.injr_241_20

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How to cite this article:
Ekbote DG, Kishor K, Sahoo RR, Wakhlu A. Macrophage activation syndrome as the presenting manifestation of systemic lupus erythematosus. Indian J Rheumatol 2021;16:115-6

How to cite this URL:
Ekbote DG, Kishor K, Sahoo RR, Wakhlu A. Macrophage activation syndrome as the presenting manifestation of systemic lupus erythematosus. Indian J Rheumatol [serial online] 2021 [cited 2021 Dec 2];16:115-6. Available from:

Dear Editor,

A 48-year-old male patient with no known co-morbidities presented with 6-week history of high-grade fever with chills and multiple joint pain. There was no history of rash, oral ulcer, photosensitivity or recent travel. On examination, he was febrile (101°F) with tender metacarpophalangeal (MCP) joints and the presence of oral candidiasis. Systemic examination was unremarkable except for mild nontender hepatomegaly. Investigations revealed leukopenia, thrombocytopenia, and raised inflammatory markers [Table 1]. He had transaminitis with normal serum bilirubin. An acute presentation with fever, arthritis, and bicytopenia necessitated screening for viral illness including parvo, chikungunya, dengue, hepatitis B surface antigen, hepatitis C virus and HIV, which were negative. Tests for malaria and typhoid were negative. Blood and urine cultures were sterile. Chest X-ray was normal, and ultrasonography of the abdomen showed mild hepatomegaly. Worsening cytopenias prompted suspicion of hemophagocytosis. Serum ferritin and lactate dehydrogenase (LDH) were done, which were markedly elevated. A diagnosis of probable hemophagocytic lymphohistiocytosis (HLH) was made, and prednisolone 0.5 mg/kg/day was started. Bone marrow examination revealed the presence of hemophagocytosis [Figure 1]a and [Figure 1]b. Possible viral triggers such as cytomegalovirus and Epstein–Barr virus were ruled out by polymerase chain reaction. The fasting serum triglyceride was normal, and fibrinogen was mildly elevated. The presence of proteinuria prompted tests for antinuclear antibody (ANA), ANA profile and anti-double-stranded DNA (dsDNA) [Table 1], which suggested a diagnosis of systemic lupus erythematosus (SLE). Hydroxychloroquine 5 mg/kg weight was started along with oral cyclosporin (3 mg/kg weight), and prednisolone was increased to 1 mg/kg. The patient gradually became apyrexic and the cytopenias normalized.
Table 1: Baseline laboratory parameters of the patient

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Figure 1: Leishman stain of bone marrow aspirate showing (a) haemophagocytosis (arrow, ×200) and (b) histiocyte engulfing neutrophil (arrowhead), lymphocyte (blue arrow), and platelets (white arrow) (×1000)

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Macrophage activation syndrome (MAS) is an accepted terminology for acquired HLH in the context of autoimmune disorders including systemic-onset juvenile idiopathic arthritis (sJIA), SLE, Kawasaki disease, adult-onset Still's disease and rheumatoid arthritis. MAS accompanying SLE has been reported and is associated with clinic-serological active disease.[1],[2] Our patient had pancytopenia and MCP arthritis along with high anti-dsDNA and low complement levels. The etiology of pancytopenia in SLE is multifactorial and at times difficult to ascertain. Besides disease activity, infections, drugs like azathioprine, MAS and secondary myelofibrosis are the important considerations.[3] Nutritional deficiencies like folate and B12 should also be ruled out. The diagnosis of MAS in SLE is challenging, as it often mimics disease flare or an infection. Elevated ferritin and LDH levels alongwith bone marrow biopsy usually help in differentiating disease activity from MAS. The diagnosis of MAS is made by modified criteria suggested by Kumakura et al., as HLH-2004 criteria are less sensitive in these patients.[4] The simultaneous diagnosis of MAS has also been reported in pediatric SLE, sJIA and Kawasaki disease.[5] High-dose steroid including pulse methyl prednisolone, cyclosporine, and intravenous immunoglobulin are used to control MAS, with the aggressive HLH protocol reserved for refractory disease.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.


We are thankful to Dr Namrata Awasthi, Professor, Department of Pathology, RML Institute of Medical Sciences, Lucknow, for providing the images of the bone marrow aspirate.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Dall'ara F, Cavazzana I, Frassi M, Taraborelli M, Fredi M, Franceschini F, et al. Six cases of macrophage activation syndrome as presenting manifestation of systemic lupus erythematosus. Ann Rheum Dis 2017;76:1225.  Back to cited text no. 1
Carvalheiras G, Anjo D, Mendonça T, Vasconcelos C, Farinha F. Hemophagocytic syndrome as one of the main primary manifestations in acute systemic lupus erythematosus-case report and literature review. Lupus 2010;19:756-61.  Back to cited text no. 2
Diaz P, Vieira MA, Carneiro A, Fernandes N. A case of pancytopenia with many possible causes: How do you tell which is the right one? Eur J Case Rep Intern Med 2019;6:001012.  Back to cited text no. 3
Kumakura S, Ishikura H, Kondo M, Murakawa Y, Masuda J, Kobayashi S. Autoimmune-associated hemophagocytic syndrome. Mod Rheumatol 2004;14:205-15.  Back to cited text no. 4
Avcin T, Tse SM, Schneider R, Ngan B, Silverman ED. Macrophage activation syndrome as the presenting manifestation of rheumatic diseases in childhood. J Pediatr 2006;148:683-6.  Back to cited text no. 5


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