Macrophage Activation Syndrome as the Initial Manifestation of Severe Juvenile Onset Systemic Lupus Erythematosus. Favorable Response to Cyclophosphamide

Jiménez, Alfonso Torres; Vallejo, Eunice Solís; Cruz, Maritza Zeferino; Cruz, Antonio Barrera; Jara, Berenice Sánchez

doi:10.1016/j.reumae.2013.12.009

Cited by 12 publications

(4 citation statements)

References 29 publications

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“…Intravenous methylprednisolone pulse therapy is considered the first-line treatment [ 30 ]; however, there has been no conclusion on which immunosuppressive agent is preferable for MAS in SLE. Several studies found that CYC and CsA had a similar effect in SLE-MAS [ 31 – 33 ]; Kumakura et al found CYC was beneficial in comparison with CsA [ 34 ]. Gavand et al found CYC or etoposide should be used for uncontrolled or severe forms in SLE-MAS [ 30 ].…”

Section: Discussionmentioning

confidence: 99%

Kikuchi-Fujimoto disease as the initial manifestation of systemic lupus erythematosus complicated with macrophage activation syndrome: two case reports and a review of literature

et al. 2022

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Background Kikuchi-Fujimoto disease (KFD) is a self-limiting and benign disease characterized by cervical lymphadenopathy and fever. Although KFD should be made differentially diagnosed from infectious, autoimmune, and malignant diseases, it sometimes occurs in patients with systemic lupus erythematosus (SLE) and can be complicated with macrophage activation syndrome (MAS). However, it is rare that KFD is the initial manifestation of SLE and to be complicated with MAS. Case presentation A 9.6-year-old girl presented with high-grade fever, double-side cervical lymphadenopathy with mild pain of one week, leukopenia, alopecia, and rash on the cheek. During hospitalization, laboratory investigations showed positive antinuclear antibody (ANA), low complement 3 (C3), and low complement 4 (C4). Imaging investigations showed pleural and pericardial effusion. A 10.3-year-old girl presented with intermittent high-grade fever, double-sided cervical lymphadenopathy with obvious pain of 1-month duration, and discoid lesion on the cheek. During hospitalization, laboratory investigations showed positive ANA, leukopenia, thrombocytopenia, anemia with positive Coombs’ test, low C3, and positive Smith antibodies. Both cases were diagnosed with KFD using lymph node biopsy, simultaneously fulfilling the diagnostic criteria of SLE. Subsequently, the two girls became complicated with MAS, followed by interstitial lung disease and neuropsychiatric lupus, respectively. Both patients benefited from high-dose methylprednisolone pulse therapy combined with intravenous cyclophosphamide. Conclusions More attention should be paid to differential diagnosis, especially SLE, in children diagnosed with KFD. In addition, children with SLE who presented with KFD as the initial manifestation seem to have a higher risk of developing MAS and experiencing organ involvement.

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Section: Discussionmentioning

confidence: 99%

Kikuchi-Fujimoto disease as the initial manifestation of systemic lupus erythematosus complicated with macrophage activation syndrome: two case reports and a review of literature

et al. 2022

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“…The therapeutic strategies for MAS in SLE are not well established and to treat this syndrome effectively, it is vital to identify the trigger. In SLE patients in which MAS is driven by disease activity in the absence of obvious infection, immunosuppressive therapy is the main treatment, including high-dose glucocorticoids, CYC or CSA [179,181]. When a flare is present along with a concomitant infection, IVIg in addition to anti-infective agents should be considered, as well as glucocorticoids [182].…”

Section: Treatmentmentioning

confidence: 99%

The diagnosis and management of the haematologic manifestations of lupus

Velo-García

Castro

Isenberg

2016

Journal of Autoimmunity

116

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Haematological manifestations in systemic lupus erythematosus (SLE) are frequently observed. They are diverse and range from mild to severe. Therefore, different treatment approaches are needed from simply keeping vigilant to significant immunosuppression. Most treatment evidence is based on case-reports or small retrospective studies, as few randomized controlled trials have been performed. The development of biological therapy has opened new possible ways to treat the most severe cases but further clinical trials are necessary. In this review we consider the most common and characteristic haematological manifestations of SLE patients, focusing on their pathogenesis and management.

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“…However, there are various cases of MAS associated with SLE flare or its complications. Very few cases of MAS have been reported as the onset of SLE [4][5][6][7][8]. Not only is this presentation rare, but it also has various diagnostic complications.…”

Section: Discussionmentioning

confidence: 99%

Macrophage Activation Syndrome Presents as Initial Manifestation of Lupus in an Adult Female

Abdelfattah,

Macpherson,

Javed

2024

Cureus

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Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by the immune system erroneously attacking healthy tissues and organs. SLE has a wide variety of clinical presentations. The signs and symptoms of SLE are very well-known, though rare presentations could occur that require early clinical attention. Macrophage activation syndrome (MAS) is a severe and life-threatening condition in which the immune system becomes overactive, leading to the excessive stimulation and proliferation of immune cells. MAS can occur as a primary immune disorder, which is not very common. It can also happen secondary to a wide variety of pathological conditions, which include infections, malignancies, autoimmune, and rheumatologic disorders. In rare cases, SLE can present with overlapping features of MAS, further complicating the clinical picture, and may require specialized management. Early recognition and intervention of this overlap are essential for improving outcomes, as delayed diagnosis and treatment can lead to significant morbidity and mortality. Here, we present a case of a young adult female who was diagnosed with SLE with the initial presentation of MAS in the form of fever, splenomegaly, cytopenia, and hemophagocytosis.

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Macrophage Activation Syndrome as the Initial Manifestation of Severe Juvenile Onset Systemic Lupus Erythematosus. Favorable Response to Cyclophosphamide

Cited by 12 publications

References 29 publications

Kikuchi-Fujimoto disease as the initial manifestation of systemic lupus erythematosus complicated with macrophage activation syndrome: two case reports and a review of literature

Kikuchi-Fujimoto disease as the initial manifestation of systemic lupus erythematosus complicated with macrophage activation syndrome: two case reports and a review of literature

The diagnosis and management of the haematologic manifestations of lupus

Macrophage Activation Syndrome Presents as Initial Manifestation of Lupus in an Adult Female

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