A Rare Case of Macrophage Activation Syndrome Presenting as the First Manifestation of Systemic Lupus Erythematosus

Poudel, Pooja; Swe, Thein; Rayancha, Sheetal

doi:10.1177/2324709618812196

Cited by 5 publications

(4 citation statements)

References 19 publications

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“…Studies have stated that macrophage activation syndrome (MAS) is more prevalent in patients with Juvenile SLE [ 30 ]. MAS is characterized by high fever, hepatosplenomegaly, hepatic dysfunction, pancytopenia, and clotting disorder.…”

Section: Discussionmentioning

confidence: 99%

YKL-40 in serum: a promising biomarker of juvenile SLE and strongly correlated with disease duration

Ali,

Yousef,

Elsheikh

et al. 2023

Ir J Med Sci

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Background The biological function of YKL-40 is not well determined in different inflammatory and autoimmune diseases; however, some data highlighted its possible connection with disease activity. Aim We investigated the diagnostic utility of serum YKL-40 in patients with SLE and examined its correlation with disease activity. Additionally, we examined any differences in serum YKL-40 levels between juvenile and adult SLE patients. Methods We included 78 female patients with SLE and 42 controls. The level of YKL-40 in serum was measured by ELISA. Results The serum YKL-40 level in SLE patients was significantly higher compared to the control group (9 (3) ng/mL vs. 5.5 (0.1) ng/mL; p < 0.001). YKL-40 showed excellent diagnostic utility with an AUC of 1 (p < 0.001) and a cutoff point of 5.6, providing sensitivity and specificity of 100%. YKL-40 was higher in adolescents and those with a positive family history of SLE (p = 0.01 for both) and positively correlated with disease duration (r = 0.45, p < 0.001). YKL-40 level was significantly higher in patients with photosensitivity, fever, vasculitis, blood disorders, positive anti-dsDNA, and APL ab (p < 0.05 for all). Conversely, patients with skin manifestations had a significantly lower YKL-40 (p = 0.004). In juvenile SLE, the AUC was 0.65 and a p-value of 0.01, and at a cutoff value of (8.7) ng/mL, the sensitivity and specificity were 72% and 60%, respectively. Conclusion YKL-40 in serum could be a promising biomarker in patients with SLE, especially in adolescent-onset cases. It is independently influenced by disease duration, anemia, thrombocytopenia, positive anti-dsDNA, and APL ab features.

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Section: Discussionmentioning

confidence: 99%

YKL-40 in serum: a promising biomarker of juvenile SLE and strongly correlated with disease duration

Ali,

Yousef,

Elsheikh

et al. 2023

Ir J Med Sci

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“…[5] The Hscore in the patient presented here was 299, which exceeded the diagnostic cutoff of 169 (Table 2). Poudel et al [6] reported a patient who presented with MAS as the initial manifestation of SLE, and concluded that early diagnosis is crucial because mortality is high in untreated cases.…”

Section: Discussionmentioning

confidence: 99%

Acute respiratory distress syndrome associated with macrophage activation syndrome in systemic lupus erythematosus

Chang

et al. 2022

Medicine

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Rationale: Previous treatment for macrophage activation syndrome (MAS) includes high-dose intravenous methylprednisolone along with intravenous immunoglobulin G. If MAS worsened, second-line therapy consisted of anakinra; if the disease remained refractory, third-line therapy with etoposide was considered. In addition, cyclosporine A plays a role in early MAS and in preventing recurrence. Some studies have reported the use of cytokine-targeting agents other than anakinra, such as canakinumab, tocilizumab, abatacept, and tofacitinib.Patient concerns: The patient with systemic lupus erythematosus (SLE) had an uncommon combination of intermittent fever, hyperferritinemia, hypertriglyceridemia, jaundice, and significantly abnormal liver function test results. The patient reported a history of daily fever of 38 to 39°C, painful oral ulcer, anorexia, abdominal bloating, diarrhea, and malar rash progression for 2 weeks, and jaundice, tea-colored urine, and clay-colored stool for 1 week preceding hospital admission.Diagnosis: SLE flareups in the patient were initially suspected. However, the final diagnosis was acute respiratory distress syndrome (ARDS) associated with MAS.Interventions: The treatment included disease-modifying antirheumatic drugs (DMARDs), such as azathioprine, and titrated steroid doses of methylprednisolone (40 mg q8 h) and dexamethasone (15 mg q8 h), after the patient had ARDS and was intubated.Dose-adjusted monotherapy with dexamethasone was found to be effective; this may be attributed to some DMARDs being unsuitable for cytokine storms, that is, some DMARDs may cause complications in cytokine storms.Outcomes: After dexamethasone 15 mg q8 h treatment, the patient's fever subsided within 2 days, and liver function became normal within 3 weeks. The patient regularly attended scheduled outpatient follow-up visits after discharge. After 2 years, the patient reported no symptoms or signs of SLE with 2 mg/d oral dexamethasone.Lessons: Early diagnosis of MAS and dexamethasone treatment for MAS with ARDS appear to be crucial for these patients.

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“…This reciprocal interaction between the initiating factors (IFNs, autoantibodies, immune complexes, infectious insult, or injury) and downstream responses (complement activation, neutrophil accumulation, and activation) likely plays an essential role in driving SLE-associated lung involvement [49]. Macrophage activation syndrome (MAS) has been described in several case reports of patients with lupus; however, its relationship is unclear and is rarely the first presentation of SLE [53][54][55]. In juvenile SLE, some cases of MAS that developed in the initial stage should be treated with immunosuppressants [56].…”

Section: Pathogenesismentioning

confidence: 99%

Systemic Lupus Erythematosus and Lung Involvement: A Comprehensive Review

Smıth

Lee

Park

et al. 2022

JCM

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Systemic lupus erythematosus (SLE) is a complex autoimmune disease with multiorgan manifestations, including pleuropulmonary involvement (20–90%). The precise mechanism of pleuropulmonary involvement in SLE is not well-understood; however, systemic type 1 interferons, circulating immune complexes, and neutrophils seem to play essential roles. There are eight types of pleuropulmonary involvement: lupus pleuritis, pleural effusion, acute lupus pneumonitis, shrinking lung syndrome, interstitial lung disease, diffuse alveolar hemorrhage (DAH), pulmonary arterial hypertension, and pulmonary embolism. DAH has a high mortality rate (68–75%). The diagnostic tools for pleuropulmonary involvement in SLE include chest X-ray (CXR), computed tomography (CT), pulmonary function tests (PFT), bronchoalveolar lavage, biopsy, technetium-99m hexamethylprophylene amine oxime perfusion scan, and (18)F-fluorodeoxyglucose positron emission tomography. An approach for detecting pleuropulmonary involvement in SLE includes high-resolution CT, CXR, and PFT. Little is known about specific therapies for pleuropulmonary involvement in SLE. However, immunosuppressive therapies such as corticosteroids and cyclophosphamide are generally used. Rituximab has also been successfully used in three of the eight pleuropulmonary involvement forms: lupus pleuritis, acute lupus pneumonitis, and shrinking lung syndrome. Pleuropulmonary manifestations are part of the clinical criteria for SLE diagnosis. However, no review article has focused on the involvement of pleuropulmonary disease in SLE. Therefore, this article summarizes the literature on the epidemiology, pathogenesis, diagnosis, and management of pleuropulmonary involvement in SLE.

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A Rare Case of Macrophage Activation Syndrome Presenting as the First Manifestation of Systemic Lupus Erythematosus

Cited by 5 publications

References 19 publications

YKL-40 in serum: a promising biomarker of juvenile SLE and strongly correlated with disease duration

YKL-40 in serum: a promising biomarker of juvenile SLE and strongly correlated with disease duration

Acute respiratory distress syndrome associated with macrophage activation syndrome in systemic lupus erythematosus

Systemic Lupus Erythematosus and Lung Involvement: A Comprehensive Review

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