Sjögren syndrome associated with protein-losing enteropathy: case-based review

Carvalho, Jozélio Freire de; Lerner, Aaron; Gonçalves, Caio Marco; Shoenfeld, Yehuda

doi:10.1007/s10067-020-05487-5

Cited by 5 publications

(2 citation statements)

References 32 publications

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“…First, TAFRO is a severe form of SS. Some cases of SS with protein-losing enteropathy have pleural ascites and generalized edema 31 ; renal dysfunction and thrombocytopenia are observed in 4% and 8.1% of SS patients, respectively 32 . Moreover, myelofibrosis has been reported in a few cases 33 .…”

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

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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%

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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“…PLE refers to the abnormally rapid loss of serum proteins into the intestinal lumen, which has been reported not only in gastrointestinal diseases but also a large number of other conditions [18,19]. Actually, previous literature reported 18 cases of PLE associated with SS [20]. Although the intestinal mucosa appears intact and there is no apparent damage in the diffusional barrier, it is assumed that there is some defect in the tight junction, which might allow free passage of molecules as large as albumin in SS [18].…”

Section: Discussionmentioning

confidence: 99%

Sjogren Syndrome Presenting as Edema and Extensive Pleural Effusion: A Pediatric Case Report

Zhang

Yin

Mao

et al. 2022

Preprint

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Background: To describe a case of pediatric Sjogren syndrome (SS) which initially presented as edema and extensive pleural effusion without sicca symptoms. Case presentation: A 16-year-old female presented to the pediatric nephrology clinic with complaint of bilateral eyelid swelling for approximately 2 months. The laboratory examinations and labial glands biopsy were consistent with SS, for which she was started on a oral prednisone regimen. Despite initial improvement, she re-presented with significantly worsened eyelid swelling and edema of the lower limbs. Abdomen distention and shifting dullness was palpable at this time. Thoracentesis was performed and analysis of hydrothorax indicated exudates. The patient also developed neurological disorder manifesting as static tremor in the course. Intravenous methylprednisolone and immunosuppression therapy was initiated. Reduction of hydrothorax and alleviation of anasarca was observed. She was able to wean off prednisone 7 months later and still remains on mycophenolate mofetil (MMF) therapy after 31 months post diagnosis. Her illness remained stable without any symptoms until now.Conclusions: Sjogren syndrome in the pediatric population may present in diverse ways in the absence of sicca symptoms. We report a case of pediatric SS, presenting with edema and extensive pleural effusion without sicca symptoms, which was successfully treated with immunosuppression. Given the wide spectrum manifestations upon disease onset, it is of great significance to improve our understanding and awareness of this presumably under-diagnosed disease.

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Prevalence and impact of Sjögren's syndrome in primary biliary cholangitis: a systematic review and meta-analysis

Deng

Hou

et al. 2022

Annals of Hepatology

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Sjögren syndrome associated with protein-losing enteropathy: case-based review

Cited by 5 publications

References 32 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

Sjogren Syndrome Presenting as Edema and Extensive Pleural Effusion: A Pediatric Case Report

Prevalence and impact of Sjögren's syndrome in primary biliary cholangitis: a systematic review and meta-analysis

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