Macrophage activation syndrome as an initial presentation of systemic lupus erythematosus with sensorineural hearing loss in a young male patient

2020 ◽  
Vol 13 (3) ◽  
pp. e233330
Author(s):  
Chou Luan Tan ◽  
Muhammad Hafizuddin Yahaya ◽  
Noor Shahrazat Ahmad ◽  
Chong Hong Lim
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Lupus Erythematosus ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Sensorineural Hearing ◽  
Systemic Lupus ◽  
Intravenous Methylprednisolone ◽  
Activation Syndrome

An 18-year-old male adolescent presented with prolonged high spiking temperature, photosensitive rash, oral ulcers and reduced hearing bilaterally of recent onset. Examination revealed malar rash, vasculitis rash over bilateral palms and soles, oral and buccal ulcers, palpable posterior auricular and inguinal lymph nodes, and reduced hearing bilaterally. His further investigations noted pancytopaenia, elevated transaminases, hyperferritinaemia, low C3 and C4 levels, positive antinuclear antibody, double-stranded DNA and direct Coombs test, while bone marrow aspiration revealed active phagocytic activity suggestive of hemophagocytic syndrome. We made a diagnosis of systemic lupus erythematosus with macrophage activation syndrome. We treated him with pulse intravenous methylprednisolone and his condition improved drastically. Temperature resolved on the next day after intravenous methylprednisolone; bilateral sensorineural hearing loss improved to near-normal hearing after treatment. He remained well during follow-up with a tapering dose of prednisolone.

Sudden Sensorineural Hearing Loss in Systemic Lupus Erythematosus and Antiphospholipid Syndrome: A Clinical Review

2020 ◽  
Vol 16 (2) ◽  
pp. 84-91
Author(s):  
Julia L. Riera ◽  
María del R. Maliandi ◽  
Jorge L. Musuruana ◽  
Javier A. Cavallasca
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Sudden Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
Clinical Feature ◽  
Systemic Lupus ◽  
Bilateral Involvement

Background: Sudden sensorineural hearing loss (SSNHL) is defined as a sudden loss of hearing, usually unilateral, of more than 30 dB in 3 contiguous frequencies of the tonal audiometry. SSNHL estimates an incidence ranging from 5 to 20 per 100.000 people per year. In approximately 75% of cases, a cause cannot be identified. However, it could be a clinical manifestation of Systemic lupus erythematosus (SLE) and Antiphospholipid Syndrome (APS). Objective: This review will focus on the clinical presentation, diagnosis, and management of the SLE and APS associated SSNHL. Methods: We searched in PubMed, Scopus, Lilacs, and Cochrane reviewing reports of Sudden sensorineural hearing loss in SLE and/or APS. Articles written in English and Spanish, and were available in full text, were included. Results: In patients with SLE, bilateral involvement was frequent. Antiphospholipid antibodies were positive in the majority of the patients. Corticosteroids were the mainstay of the treatment. The auditory prognosis was poor with total hearing loss recovery reached in only 22% of patients. : On the other hand, most of the patients with SSNHL and APS were males and presented associated symptoms such as vertigo, tinnitus and/or headache, 75% had bilateral disease. Lupus anticoagulant and aCL were found in equal proportions, all patients were anticoagulated, and aspirin was associated in 25% of the cases. Complete resolution or improvement of symptoms was observed in 25% of the patients. Conclusion: Sudden sensorineural hearing loss, can be a clinical feature of SLE and APS. Treating physicians should be aware of this devastating complication, especially when bilateral involvement occurs.

scholarly journals Arthritis and use of hydroxychloroquine associated with a decreased risk of macrophage activation syndrome among adult patients hospitalized with systemic lupus erythematosus

Lupus ◽  
2018 ◽  
Vol 27 (7) ◽  
pp. 1065-1071 ◽  
Author(s):  
E M Cohen ◽  
K D’Silva ◽  
D Kreps ◽  
M B Son ◽  
K H Costenbader
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Hospital Admission ◽  
Lupus Erythematosus ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Activity Index ◽  
Conditional Logistic Regression ◽  
Systemic Lupus ◽  
Factors Associated ◽  
Activation Syndrome

Background Macrophage activation syndrome (MAS) is an uncommon but serious complication of systemic lupus erythematosus (SLE). We aimed to identify factors associated with MAS among adult hospitalized SLE patients. Methods Within the Brigham and Women’s Hospital (BWH) Lupus Center Registry, we identified adult SLE patients > age 17 who had been hospitalized from 1970 to 2016, with either ferritin > 5000 ng/ml during admission or “macrophage activation syndrome” or “MAS” in discharge summary. We confirmed MAS by physician diagnosis in medical record review. We matched each hospitalized SLE patient with MAS to four SLE patients hospitalized without MAS (by SLE diagnosis date ±1 year). We employed conditional logistic regression models to identify clinical factors associated with MAS among hospitalized SLE patients. Results Among 2094 patients with confirmed SLE, we identified 23 who had a hospitalization with MAS and compared them to 92 hospitalized without MAS. Cases and controls had similar age at SLE diagnosis (29.0 vs. 30.5, p = 0.60), and hospital admission (43.0 vs. 38.3, p = 0.80), proportion female (78% vs. 84%, p = 0.55), and time between SLE diagnosis and hospitalization (1971 vs. 1732 days, p = 0.84). Arthritis (OR 0.04 (95% CI 0.004–0.35)) and hydroxychloroquine use (OR 0.18 (95% CI 0.04–0.72)) on admission were associated with decreased MAS risk. Admission Systemic Lupus Erythematosus Disease Activity Index scores (30 vs. 19, p = 0.002) and lengths of stay (16 days vs. 3 days, p < 0.0001) were much higher among cases. Death during hospitalization was 19% among cases and 3% among controls ( p = 0.03). Conclusions In this case-control study of hospitalized adult SLE patients, arthritis and hydroxychloroquine use at hospital admission were associated with decreased MAS risk. Further studies are needed to validate these factors associated with lowered MAS risk.

Effect of systemic lupus erythematosus and rheumatoid arthritis on sudden sensorineural hearing loss

2019 ◽  
Vol 130 (10) ◽  
pp. 2475-2480 ◽  
Author(s):  
Shaobing Xie ◽  
Hongting Ning ◽  
Yongchuan She ◽  
Qiancheng Jing ◽  
Qian Jiang ◽  
...  
Keyword(s):  
Rheumatoid Arthritis ◽  
Systemic Lupus Erythematosus ◽  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Lupus Erythematosus ◽  
Sudden Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
Systemic Lupus

scholarly journals Macrophage Activation Syndrome, Glomerulonephritis, Pericarditis, and Retinal Vasculitis as Initial Presentation of Systemic Lupus Erythematosus

2018 ◽  
Vol 2018 ◽  
pp. 1-5
Author(s):  
Yiming Luo ◽  
Yumeng Wen ◽  
Ana Belen Arevalo Molina ◽  
Punya Dahal ◽  
Lorenz Leuprecht ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Retinal Vasculitis ◽  
Initial Presentation ◽  
Systemic Lupus ◽  
First Case ◽  
Multisystem Involvement ◽  
Activation Syndrome

Macrophage activation syndrome (MAS) is a rare manifestation of systemic lupus erythematosus (SLE) with potentially life-threatening consequences. To the best of our knowledge, this is the first case reported in literature for a constellation of MAS, glomerulonephritis, pericarditis, and retinal vasculitis as initial presentation of SLE. Despite extensive multisystem involvement of his disease, the patient responded well to initial steroid treatment, with mycophenolate mofetil successfully added as a steroid-sparing agent. Our case highlights the importance of multispecialty collaboration in the diagnosis and management of SLE with multisystem involvement.

UnMASking the diagnosis: Acute severe necrotizing pancreatitis in the setting of systemic lupus erythematosus complicated by macrophage activation syndrome

2018 ◽  
Vol 5 (4) ◽  
pp. 1
Author(s):  
Cyrus Ashraf Askin ◽  
Jerome Craig Edelson ◽  
Guy Smith Dooley ◽  
Amy Nicole Stratton
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Acute Pancreatitis ◽  
Inflammatory Response ◽  
Lupus Erythematosus ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Necrotizing Pancreatitis ◽  
Adult Onset ◽  
Systemic Lupus ◽  
Activation Syndrome

Systemic lupus erythematosus (SLE) is a common rheumatologic condition with known GI involvement. Acute pancreatitis (AP) is a rare GI complication of SLE and is typically associated with increased disease activity. Macrophage activation syndrome (MAS) is an unusual, hyper-inflammatory response to a rheumatologic stimulus characterized by hyperferritinemia, pancytopenia, thermal dysregulation and multi-organ dysfunction. MAS, more commonly seen in children, has been reported to complicate both adult onset SLE and AP. We present a case of necrotizing AP secondary to an SLE flare complicated by MAS in an adult patient successfully treated with anakinra.

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