Intractable Hemophagocytic Syndrome Associated with Systemic Lupus Erythematosus Resistant to Corticosteroids and Intravenous Cyclophosphamide That Was Successfully Treated with Cyclosporine A

Toko, Hirofumi; Tsuboi, Hiroto; Umeda, N.; Honda, Fumika; Ohyama, Ayako; Takahashi, Hidenori; Abe, Saori; Yokosawa, Masahiro; Asashima, Hiromitsu; Hagiwara, Shinji; Hirota, Tomoya; Kondo, Yuya; Matsumoto, Isao; Sumida, Takayuki

doi:10.2169/internalmedicine.0571-17

Cited by 3 publications

(1 citation statement)

References 18 publications

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“…Hemophagocytic lymphohistiocytosis (HLH) represents a rare and life-threatening condition in which a pro-inflammatory state leads to uncontrolled macrophage and T-cell activation with widespread hemophagocytosis, culminating in multisystemic dysfunction [ 5 , 6 ]. The association between SLE and Hemophagocytic Syndrome is rare and represents a challenging situation with a reserved prognosis [ 7 ].…”

Section: Introductionmentioning

confidence: 99%

Post-partum Fever of Unknown Origin: An Inaugural Flare of Severe Lupus With Multisystemic Involvement and Hemophagocytic Syndrome

Fernandes¹,

Ferreira²,

Lynce³

et al. 2023

Cureus

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Systemic lupus erythematosus (SLE) is an autoimmune disease that can affect almost every organ. Lupus protein-losing enteropathy (PLE) is one of the rarest manifestations of gastrointestinal involvement. Lupus flare as initial presentation is rare and the disease can act as a trigger to other pathologic immune syndromes like Hemophagocytic Lymphohistiocytosis (HLH), although this association is rare.We report the case of a previously healthy African 39-year-old female patient, with a recent history of cesarean section. Admitted to the Emergency Department (ED) with diffuse abdominal pain and fever, having completed a cycle of antibiotic therapy for initially suspected endometritis. The clinical picture progressed with sustained high fever, new-onset lymphadenopathies, systemic rash, acute pulmonary edema and seizures. Laboratory findings included hyperferritinemia, hypertriglyceridemia, proteinuria and hypoalbuminemia. The auto-immune panel was positive for antinuclear antibodies (ANA), anti-dsDNA, anti-SSA and anti-SSB, anti-PL7, anti-RNP, anti-U1-SnRNP, and anti-Pm-Scl75. She also presented hypocomplementemia. An inaugural flare of SLE with multisystemic involvement and concomitant secondary Hemophagocytic Syndrome was considered and therapy with methylprednisolone pulses, Anakinra and Cyclophosphamide was started. By the end of the first cycle of cyclophosphamide, the patient presented clinical worsening with abdominal pain recrudescence and profuse diarrhea. After the exclusion of an infectious process, a Lupus PLE was assumed and Cyclophosphamide protocol was resumed, with sustained clinical improvement after the induction protocol.Despite initially suspected gynecological infection, the clinical progression with multisystemic involvement together with the auto-immune panel made the diagnosis of SLE possible, with other laboratory findings raising the suspicion of HLH. This case represents a rare report of severe SLE with multiple organ involvement accompanied by HLH. Gastrointestinal involvement with PLE added rarity and morbidity to the clinical picture. The case reinforces the idea that when organ dysfunction is due to a severe autoimmune response, supportive treatment can be lifesaving until immunosuppressive drugs reach their full effect.

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Section: Introductionmentioning

confidence: 99%

Post-partum Fever of Unknown Origin: An Inaugural Flare of Severe Lupus With Multisystemic Involvement and Hemophagocytic Syndrome

Fernandes¹,

Ferreira²,

Lynce³

et al. 2023

Cureus

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Vitamin D Activates miR-126a-5p to Target GSK-3β and Alleviates Systemic Lupus Erythematosus in MRL/LPR Mice

Min-shu

Song

Yin

et al. 2023

CPB

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Background: The etiology of systemic lupus erythematosus (SLE) is complex, and the disease is thus difficult to cure. In this regard, it has been established that SLE patients are characterized by differing levels of vitamin D-hydroxylation; however, the direct effects of vitamin D (VitD) in these patients remain unknown. Objective: Therefore, we investigated the effects and mechanisms of action of VitD in the context of SLE. Methods: The effects of VitD on MRL/LPR mice were studied by synthesizing glycogen synthase kinase-3β (GSK-3β)-interfering lentiviruses and transfecting with miR-126a-5p mimics. Changes in the body weight of mice were recorded for 6 weeks. Western blotting was performed to determine the levels of T-bet, GATA3, and GSK-3β protein expression, and qRT-PCR was performed to determine the levels of miR-126a-5p and GSK-3β mRNA expression. ELISA was performed to determine the levels of ANA, dsDNA, and snRNP/Sm in mice serum. method: The effect of VitD on MRL/LPR mice was studied by synthesizing GSK-3β-interfering lentivirus and transfecting miR-126a-5p mimics. Body weight changes were recorded for 6 weeks. Western blot was performed to detect T-bet, GATA3, and GSK-3β protein expression levels, and qRT-PCR was performed to detect miR-126a-5p and GSK-3β mRNA expression levels. ELISA was performed to detect ANA, dsDNA, snRNP/Sm levels in serum. Results: GSK-3β and miR-126a-5p were expressed at high and low levels, respectively, in MRL/LPR mice. VitD (30 ng/kg) was found to reduce the expression of GSK-3β and increase miR-126a-5p expression, which targets GSK-3β. T-bet and GATA3 were found to be positively regulated by miR-126a-5p and VitD and negatively regulated by GSK-3β. The body weight of mice was not altered by VitD. ANA, dsDNA, and snRNP/Sm were positively regulated by miR-126a-5p and VitD and negatively regulated by GSK-3β. The effects of GSK-3β were enhanced in response to the inhibition of miR-126a-5p expression. Conclusion: VitD upregulated miR-126a-5p to target GSK-3β expression, thereby alleviating the SLE in MRL/LPR mice. other: none

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Systemic Lupus Erythematosus: Review of Modern Methods of Diagnosis, Treatment and Description of the Clinical Case

et al. 2021

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A clinical case of systemic lupus erythematosus (SLE) is presented. Since childhood the patient had preclinical lupus: leukopenia, anemia, photoallergy, vasculitis, and serological changes a-SS-A (+++), but at that time the findings did not meet the criteria for SLE. At the initial examination of the woman at the age of 24 years, a clinical blood test revealed hemoglobin 101 g/l, leukopenia 3.2×109/l, ESR 41 mm/h, hyper-γ-globulinemia 29%. The immunological blood analysis revealed a-DNA (+++), a-SS-A (+++), aSm (+++), aRNP (+++). The diagnosis was "Systemic lupus erythematosus of chronic (at the beginning) course, activity of the 1st degree (2 points by the SLEDA scale): with hematological disorders (leukopenia), immunological disorders (ANA+)". Metypred was administered at a dose of 16 mg/day, followed by the dose reduction to 6 mg/day and Plaquenil 400 mg/day. The woman received inpatient and outpatient treatment, repeatedly consulted in the Federal State Budgetary Institution "V.A. Nasonova Research Institute" under the Russian Academy of Medical Sciences. At the age of 26, the diagnosis was made: "Systemic lupus erythematosus, grade 2 activity with skin lesions of the type of subacute cutaneous lupus, cheilitis, lymphadenopathy", blood tests revealed leukopenia, lymphopenia, anemia, as well as immunological disorders: a-n DNA 93.4 u/ml, aSm > 200 u/ml, aRo > 200 u/ml, C3 0.63 g/l, rheumatoid factor 69.5 mIU/ml, ANA (Нер-2) 1/640 Sp. A complex therapy was performed with the use of Metypred, Cyclophosphane and Azathioprine. Against the background of the therapy, the patient's condition is stable, but immunological disorders are preserved: persistent high positivity for aRo-SS-A and the presence of the rheumatoid factor. The case demonstrates the need for an in-depth examination of women with anemia of unknown origin in combination with leukopenia to exclude systemic diseases of the connective tissue.

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Intractable Hemophagocytic Syndrome Associated with Systemic Lupus Erythematosus Resistant to Corticosteroids and Intravenous Cyclophosphamide That Was Successfully Treated with Cyclosporine A

Cited by 3 publications

References 18 publications

Post-partum Fever of Unknown Origin: An Inaugural Flare of Severe Lupus With Multisystemic Involvement and Hemophagocytic Syndrome

Post-partum Fever of Unknown Origin: An Inaugural Flare of Severe Lupus With Multisystemic Involvement and Hemophagocytic Syndrome

Vitamin D Activates miR-126a-5p to Target GSK-3β and Alleviates Systemic Lupus Erythematosus in MRL/LPR Mice

Systemic Lupus Erythematosus: Review of Modern Methods of Diagnosis, Treatment and Description of the Clinical Case

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