Severe TAFRO Syndrome Mimicking Hepatorenal Syndrome Successfully Treated with a Multidisciplinary Approach: A Case Report and Literature Review

Yamamoto, Shinya; Wells, Ken; Morita, Keisuke; Tanigaki, Katsuya; Muro, Koji; Matsumoto, Minami; Nakai, Hirotsugu; Arai, Yasuyuki; Akizuki, Shuji; Takahashi, Ken; Minamiguchi, Sachiko; Fukuma, Shingo; Yanagita, Motoko

doi:10.2169/internalmedicine.1178-22

Cited by 3 publications

(3 citation statements)

References 26 publications

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“…In recent years, there have been many reports on the relationship between TAFRO and anti-SSA antibodies, and some patients have been diagnosed with both SS and TAFRO 7 , 17 – 23 . In addition, in some cases, patients tested positive for anti-SSA antibodies but did not undergo lip biopsy or oral or ophthalmological examination because of the absence of symptoms of dryness 24 – 29 . Some of them were also positive for anti-SSB antibodies, which are highly specific for SS 8 , 29 .…”

Section: Discussionmentioning

confidence: 99%

“…In addition, in some cases, patients tested positive for anti-SSA antibodies but did not undergo lip biopsy or oral or ophthalmological examination because of the absence of symptoms of dryness 24 – 29 . Some of them were also positive for anti-SSB antibodies, which are highly specific for SS 8 , 29 . In cases with a confirmed diagnosis of SS, TAFRO and SS were diagnosed simultaneously, or SS preceded TAFRO 30 .…”

Section: Discussionmentioning

confidence: 99%

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TAFRO syndrome is associated with anti-SSA/Ro60 antibodies, in contrast to idiopathic castleman disease

Shirakashi,

Nishida,

Nakashima

et al. 2024

Sci Rep

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TAFRO syndrome is an acute systemic inflammatory disease characterized by thrombocytopenia, anasarca, fever, reticulin fibrosis/renal dysfunction, and organomegaly. There have been increasing reports that TAFRO is a disease distinct from idiopathic multicentric Castleman disease and that TAFRO patients may be positive for anti-SSA antibodies. To assess anti-SSA antibody positivity and the clinical characteristics of the two diseases, we retrospectively compared 7 TAFRO and 10 iMCD patients in our hospital. The mean age of onset of TAFRO and iMCD was 48.0 (interquartile range [IQR], 41–53) and 45.0 (IQR, 35–53) years, respectively. The TAFRO and iMCD groups had 6 (86%) and 4 (40%) male patients, respectively, and the following pretreatment laboratory values: platelet count, 3.8 (IQR, 2.2–6.4) and 35.5 (IQR, 22.2–42.8) × 104/μL, respectively; C-reactive protein, 10.2 (IQR, 6.8–21.4) and 9.5 (IQR, 6.2–13.6) mg/dL, respectively; IgG, 1431 (IQR, 1112–1815) and 4725 (IQR, 3755–5121) mg/dL, respectively. RNA immunoprecipitation (5 cases for anti-SSA) or protein array (5 cases for anti-SSA/Ro60) detected anti-SSA antibodies in six (86%) TAFRO patients but not in iMCD patients; it did not detect anti-SSB antibodies in any of the patients. None of the patients were diagnosed with Sjögren syndrome. All iMCD patients treated with tocilizumab (TCZ) responded well. Meanwhile, two of six TAFRO patients treated with TCZ showed inadequate responses; thus, both patients were switched to rituximab, following which they achieved remission. TAFRO and iMCD have different clinical features. TAFRO may be categorized as a severe phenotype of the anti-SSA antibody syndrome.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

TAFRO syndrome is associated with anti-SSA/Ro60 antibodies, in contrast to idiopathic castleman disease

Shirakashi,

Nishida,

Nakashima

et al. 2024

Sci Rep

Self Cite

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“…Bacterial infection [29] Toxic epidermal necrolysis [33] Cyclosporine A Hepatotoxicity [20,30,[34][35][36] Renal toxicity [37] Thrombotic microangiopathy [38] Combination therapy with glucocorticoid Tocilizumab Bacterial infection [20,[39][40][41][42][43] CMV infection [37,[43][44][45][46] Fungal infection [40,42] Cyclosporine A…”

Section: Tocilizumabmentioning

confidence: 99%

Tacrolimus Treatment for TAFRO Syndrome

Shirai,

Ichikawa,

Saegusa

2024

Biomedicines

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TAFRO syndrome is an acute systemic inflammatory disorder characterized by thrombocytopenia, anasarca, fever, reticulin myelofibrosis, renal dysfunction, and organomegaly. While its lymph node pathology is similar to that of idiopathic multicentric Castleman disease (iMCD), the clinical features of TAFRO syndrome differ from those of typical iMCD, as they include a more aggressive clinical course and high mortality. However, an optimal treatment strategy for TAFRO syndrome has not yet been established, owing to a poor understanding of its pathogenesis. The limited cases we encountered suggest that tacrolimus treatment in combination with glucocorticoids may potentially be effective and well tolerated as an initial treatment, and hold promise as a glucocorticoid-sparing agent. Herein, we report an additional case and review the sparse literature available regarding TAFRO syndrome treated via tacrolimus.

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Multiple drugs

2023

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Severe TAFRO Syndrome Mimicking Hepatorenal Syndrome Successfully Treated with a Multidisciplinary Approach: A Case Report and Literature Review

Cited by 3 publications

References 26 publications

TAFRO syndrome is associated with anti-SSA/Ro60 antibodies, in contrast to idiopathic castleman disease

TAFRO syndrome is associated with anti-SSA/Ro60 antibodies, in contrast to idiopathic castleman disease

Tacrolimus Treatment for TAFRO Syndrome

Multiple drugs

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