Cytokine profile in two siblings with neonatal lupus erythematosus

Hironori, Shimozawa; Kono, Yoshiaki; Matano, Miyuki; Suzuki, Yume; Koike, Yasunori; Yada, Yukari; Yamagata, Takanori; Takahashi, Naoto

doi:10.1111/ped.12723

Cited by 10 publications

(9 citation statements)

References 9 publications

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“…11 Tanasescu et al 12 demonstrated that LE patients with serum anti-SSA antibodies had more IL-17A-positive lymphocytes in the skin compared to those without anti-SSA antibodies. In addition, Shimozawa et al 13 reported a neonatal LE case, which showed the typical eruptions and elevated serum IL-17. These results suggest a pathogenic association between anti-SSA antibody and IL-17-mediated inflammation in neonatal LE, possibly by Th17 activation via dendritic cells.…”

Section: Discussionmentioning

confidence: 99%

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Neonatal lupus erythematosus exacerbated by vaccination

Ishikawa

Mori

Yamamoto

2020

The Journal of Dermatology

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Neonatal lupus erythematosus (LE) is a rare immune-mediated disease caused by placental transport of maternal anti-SSA/Ro, anti-SSB/La and/or anti-U1RNP antibodies. Here, we demonstrate two cases of neonatal LE, in both of which cutaneous LE was exacerbated by inoculation. To our knowledge, cases worsening neonatal LE after administration of vaccines have not been reported. In case 1, not only exacerbation of pre-existing annular erythema but also spreading of new erythematous lesions to the trunk and extremities were induced following vaccination. Of interest, all of the lesions simultaneously improved. By contrast, in case 2, pre-existing facial erythema became prominent without spreading to other sites. The mother of case 1 had Sj€ ogren's syndrome, whereas in case 2, the mother was diagnosed with Sj€ ogren's syndrome on this occasion for the first time. Immunohistochemistry in case 1 revealed interleukin (IL)-17-positive cells infiltrating into the papillary dermis, and CD123-positive plasmacytoid dendritic cells in the papillary dermis and the deep reticular dermis. Both innate immune response and IL-17 mediated inflammation following vaccination are speculated as a possible mechanism of the deterioration of LE lesions in our juvenile cases. Caution is necessary since neonatal LE can be worsened following vaccination.

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Section: Discussionmentioning

confidence: 99%

“…demonstrated that LE patients with serum anti‐SSA antibodies had more IL‐17A‐positive lymphocytes in the skin compared to those without anti‐SSA antibodies. In addition, Shimozawa et al 13 . reported a neonatal LE case, which showed the typical eruptions and elevated serum IL‐17.…”

Section: Discussionmentioning

confidence: 99%

Neonatal lupus erythematosus exacerbated by vaccination

Ishikawa

Mori

Yamamoto

2020

The Journal of Dermatology

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“…According to the literature, 6-65% of patients with HLH have skin involvement during the course of disease [3,5,24]. Also in MAS/HLH, skin findings have been reported [7,25,26]. Cutaneous manifestations of MAS/HLH are however aspecific as most patients present with a transient generalized maculopapular eruption.…”

Section: Skin Lesionsmentioning

confidence: 99%

“…It has rarely been associated with neonatal lupus. So far, only four patients have been reported in the literature (Table 1) [6][7][8][9].…”

Section: Introductionmentioning

confidence: 99%

Macrophage activation syndrome in a newborn: report of a case associated with neonatal lupus erythematosus and a summary of the literature

et al. 2021

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Background Macrophage activation syndrome (MAS) is a life-threatening hyperinflammatory syndrome and is caused by a severely dysregulated immune response. It has rarely been associated with neonatal lupus. Case presentation We present a female neonate with MAS born to a mother who had cutaneous lupus erythematosus with circulating anti-nuclear antibodies (ANA), anti-SSA, anti-SSB and anti-extractable nuclear antigen (anti-ENA) antibodies. Because of neonatal lupus (NLE) with a total atrioventricular block, epicardial pacemaker implantation was required on the sixth day of life. Following surgery she developed non-remitting fever and disseminated erythematous skin lesions. A diagnosis of MAS was made based on these symptoms, with hyperferritinemia, elevated transaminases, hypertriglyceridemia, and a skin biopsy that showed hemophagocytosis. Our patient was treated with steroids for 3 months with good effect. No relapse has occurred. Conclusions MAS is a rare complication of neonatal lupus that may be difficult to diagnose, but needs to be treated promptly. In this article, pathogenesis and overlap of MAS and hemophagocytic lymphohistiocytosis (HLH) has been described. Diagnosis of MAS can be difficult. Different diagnostic criteria are used in both diagnosing MAS and HLH. Validated criteria for diagnosis of MAS in other disease than systemic onset JIA have not been validated yet. In NLE, diagnosing MAS is even more difficult, since skin lesions are already common in NLE. We show the potential additional value of skin biopsy in diagnosing MAS.

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“…A recent study found increased frequencies of IFNγ‐producing cells (both NK and T cells) in cord blood from neonates exposed to anti‐Ro/La antibodies, 83 suggesting that a general activation of innate immune mechanisms may not be restricted to autoimmune CHB cases. In fact, hypercytokinemia has been reported, not only in a few cases of autoimmune CHB or non‐cardiac NLE, 87 but also in babies born to mothers with other systemic rheumatic diseases 69 . An interesting hypothesis is that foetuses exposed to maternal anti‐Ro/La antibodies and/or type I IFN have an activated, pro‐inflammatory immune profile that facilitates the development of CHB manifestations brought about by additional events and/or susceptibility factors.…”

Section: Time To Look Beyond Macrophage‐driven Inflammation and Fibromentioning

confidence: 99%

Interferons and innate immune activation in autoimmune congenital heart block

et al. 2020

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Autoimmune congenital heart block (CHB) may develop in foetuses of women carrying anti-Ro/SSA and La/SSB autoantibodies and is characterized by disruption of signal conduction at the atrioventricular (AV) node, resulting in partial or complete AV block. If not fatal in utero, complete CHB typically requires lifelong cardiac pacing. No treatment has so far been unequivocally demonstrated to prevent or treat autoimmune CHB, and the relatively low incidence (1%-5%) and recurrence (12%-16%) rates of second/third-degree AV block add to the complexity of managing pregnancies in women with anti-Ro/La antibodies. Altogether, a better understanding of events leading to development of autoimmune CHB is needed to improve surveillance and treatment strategies. In the past decade, studies have started to look beyond the role of maternal autoantibodies in disease pathogenesis to assess other contributing factors such as foetal genetics and, more recently, immune responses in foetuses and neonates of anti-Ro/La antibody-positive women. In this review, we provide an update on the epidemiology, clinical presentation and current treatment approaches of autoimmune CHB, summarize the previously proposed pathogenic mechanisms implicating maternal autoantibodies, and discuss the recent findings of type I interferon (IFN) and innate immune activation in foetuses with autoimmune CHB and in neonates of anti-Ro/La antibody-positive mothers, and how these may contribute to autoimmune CHB pathogenesis.

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Cytokine profile in two siblings with neonatal lupus erythematosus

Cited by 10 publications

References 9 publications

Neonatal lupus erythematosus exacerbated by vaccination

Neonatal lupus erythematosus exacerbated by vaccination

Macrophage activation syndrome in a newborn: report of a case associated with neonatal lupus erythematosus and a summary of the literature

Interferons and innate immune activation in autoimmune congenital heart block

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