Macrophage activation syndrome triggered by cytomegalovirus in a patient with granulomatosis with polyangiitis

Aslan, Bengisu; Erbasan, Funda; Öğüt, Tahir; Dilbil, Melis; Yazısız, Veli; Terzıoğlu, Ender

doi:10.1093/rheumatology/keab778

Cited by 4 publications

(1 citation statement)

References 7 publications

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“…If the condition is serious and the condition is still not controlled after the above treatment, a reduced etoposide frequency should be considered [7]. In addition, IVIG may be useful in treatment [8]. Our reported case was effectively controlled with MAS after high-dose glucocorticoid, IVIG, and etoposide treatment.…”

Section: Discussionmentioning

confidence: 74%

Macrophage Activation Syndrome Originating from Primary Biliary Cholangitis: A Case Report

Ren

2023

IJCCR

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A 49-year-old female had intermittent fever for one year, with maximum recorded temperature of 39.0℃, accompanied by itchy skin, no joint swelling and pain, ineffective in antibiotic treatment, and responding well to non-steroidal anti-inflammatory drugs (NSAIDs). Patchy rash appeared on the trunk during fever, which faded away when the fever subsided. One month ago, she developed hyperpyrexia again, complicated by anorexia. Pertinent laboratory findings were as follows: hemoglobin, 98 g/L; aspartate aminotransferase (AST), 806 U/L; alanine aminotransferase (ALT), 644 U/L; alkaline phosphatase (ALP), 279 U/L; gamma-glutamyl transferase (GGT), 115U/L; C-reactive protein, 77.04 mg/L; erythrocyte sedimentation rate (ESR), 58mm/h; ferritin, 6415 ng/mL; anti-nuclear Ab (ANA), speckled pattern, 320(+); anti-M2 Ab (AMA-M2), 41.27 units (+). Infection and tumor screening showed no obvious abnormality.

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Section: Discussionmentioning

confidence: 74%

Macrophage Activation Syndrome Originating from Primary Biliary Cholangitis: A Case Report

Ren

2023

IJCCR

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Secondary haemophagocytic lymphohistiocytosis in a patient with new-onset systemic lupus erythematosus: the challenges of timely diagnosis and successful treatment

et al. 2023

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Haemophagocytic lymphohistiocytosis (HLH) is an immune-mediated disease driven by abnormal macrophage activation and regulatory cell dysfunction. HLH can be primary due to genetic mutations or secondary due to infection, malignancy or autoimmune conditions. We describe a woman in her early 30s who developed HLH while being treated for newly diagnosed systemic lupus erythematosus (SLE) complicated by lupus nephritis as well as concomitant cytomegalovirus (CMV) reactivation from a dormant infection. The trigger for this secondary form of HLH may have been either aggressive SLE and/or CMV reactivation. Despite prompt treatment with immunosuppressive therapies for SLE consisting of high-dose corticosteroids, mycophenolate mofetil, tacrolimus, etoposide for HLH and ganciclovir for CMV infection, the patient developed multiorgan failure and passed away. We demonstrate the difficulty in identifying a specific cause for secondary HLH when multiple conditions are present (SLE and CMV) and the fact that, despite aggressive treatment for both conditions, the mortality for HLH remains high.

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Heterogeneity of macrophage activation syndrome and treatment progression

Dong,

Wang,

2024

Front. Immunol.

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Macrophage activation syndrome (MAS) is a rare complication of autoimmune inflammatory rheumatic diseases (AIIRD) characterized by a progressive and life-threatening condition with features including cytokine storm and hemophagocytosis. Predisposing factors are typically associated with microbial infections, genetic factors (distinct from typical genetically related hemophagocytic lymphohistiocytosis (HLH)), and inappropriate immune system overactivation. Clinical features include unremitting fever, generalized rash, hepatosplenomegaly, lymphadenopathy, anemia, worsening liver function, and neurological involvement. MAS can occur in various AIIRDs, including but not limited to systemic juvenile idiopathic arthritis (sJIA), adult-onset Still’s disease (AOSD), systemic lupus erythematosus (SLE), Kawasaki disease (KD), juvenile dermatomyositis (JDM), rheumatoid arthritis (RA), and Sjögren’s syndrome (SS), etc. Although progress has been made in understanding the pathogenesis and treatment of MAS, it is important to recognize the differences between different diseases and the various treatment options available. This article summarizes the cell types and cytokines involved in MAS-related diseases, the heterogeneity, and treatment options, while also comparing it to genetically related HLH.

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Macrophage activation syndrome triggered by cytomegalovirus in a patient with granulomatosis with polyangiitis

Cited by 4 publications

References 7 publications

Macrophage Activation Syndrome Originating from Primary Biliary Cholangitis: A Case Report

Macrophage Activation Syndrome Originating from Primary Biliary Cholangitis: A Case Report

Secondary haemophagocytic lymphohistiocytosis in a patient with new-onset systemic lupus erythematosus: the challenges of timely diagnosis and successful treatment

Heterogeneity of macrophage activation syndrome and treatment progression

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