Predicting the risk for lymphoma development in Sjogren syndrome

Fragkioudaki, Sofia; Mavragani, Clio P.; Moutsopoulos, Haralampos Μ.

doi:10.1097/md.0000000000003766

Cited by 159 publications

(150 citation statements)

References 48 publications

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“…Es importante saber que 5% de los pacientes pueden desarrollar linfoma, mayormente MALT y ocasionalmente grande difuso. Un marcador clínico de riesgo para esto es el agrandamiento parotídeo progresivo (2). Llamaba la atención que la paciente no tenía síntomas de xeroftalmia y que su serología de autoinmunidad era negativa.…”

Section: Gran Ronda De Medicina Interna Y Especialidades Del Hospitalunclassified

Caso clínico 01-2017. Mujer de 20 años con crecimiento de glándulas salivares y lacrimales, adenopatías, fiebre y lesión pulmonar

Vásquez¹,

Mejía²,

Rodriguez³

et al. 2017

Rev Med Hered

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Mujer de 20 años, natural de Iquitos y procedente de Lima quien hace 4 semanas presentó aumento de volumen en el borde inferior del párpado superior izquierdo y una semana después del párpado superior derecho. Dos semanas antes del ingreso se agregó crecimiento parotídeo bilateral y en región submaxilar y una semana antes de la admisión se añadió otalgia bilateral, disfonía y xerostomía. Durante esa semana también notó aparición de un ganglio axilar derecho, no doloroso, sensación de alza térmica y tos no productiva por lo cual acudió al hospital, siendo hospitalizada. En las funciones biológicas tenía hiporexia, sed aumentada, sudoración normal y refería una pérdida de peso de 4 kg en el último mes. Las deposiciones y orina no mostraban alteraciones y estaba eutímica.La paciente vivía en Lima hace varios años y era estudiante de administración. No ingería alcohol no fumaba ni usaba drogas. Su historia ginecológica era G: 0 P: 0, régimen catamenial 5/30, negaba relaciones sexuales y su FUR fue dos semanas antes del ingreso.No tenía antecedentes patológicos de importancia y previamente había estado sana. No había tenido intervenciones quirúrgicas. No tenía alergias a medicamentos. No tenía hermanos y sus padres no sufrían de enfermedades. En la revisión anamnésica de sistemas y aparatos negaba xeroftalmia, alopecia, artralgias o lesiones dérmicas.Al examen físico la PA: 100/60 mmHg FC: 78 x' FR: 18 x' T: 36,4°C; SAT: 97%; FiO 2 : 21%; peso: 54 kg; talla: 157 cm; IMC: 22,4. Lucía crónicamente enferma en regular estado general, hidratada, lúcida y orientada en las tres esferas, llamó la atención su fascies (Figura 1). La piel, tejido celular subcutáneo y sistema osteomioarticular no mostraban alteraciones significativas. Se palpaba ganglio axilar derecho de 2 cm, no doloroso, móvil. Al examen regional se evidenció crecimiento de glándulas lacrimales, parótidas y glándula salivar submaxilar; el resto del cuello y las mamas, sin alteraciones. En tórax el murmullo vesicular pasaba bien en ambos campos pulmonares y no se auscultaron rales. En el examen cardiovascular, los ruidos cardiacos eran rítmicos, no habían soplos y los pulsos estaban conservados; no había ingurgitación yugular. El abdomen era plano, los ruidos hidroaéreos estaban presentes, y no se palpaban masas ni vísceromegalia. Al examen neurológico las

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Section: Gran Ronda De Medicina Interna Y Especialidades Del Hospitalunclassified

Caso clínico 01-2017. Mujer de 20 años con crecimiento de glándulas salivares y lacrimales, adenopatías, fiebre y lesión pulmonar

Vásquez¹,

Mejía²,

Rodriguez³

et al. 2017

Rev Med Hered

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“…The risk of developing non-Hodgkin's lymphoma in SS is well documented, and associations with other haematological malignancies, although uncommon, have been described [8,9]. Here, we report on two cases of the concomitant occurrence of acute leukaemia (AL) and suspected SS, and review the literature on this topic.…”

Section: Introductionmentioning

confidence: 96%

Unusual association of two cases of acute myeloblastic leukaemia and possible Sjogren’s syndrome and review of literature

Brault¹,

Gourguechon²,

Chehimi³

et al. 2017

Hematol Leuk

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“…REFERENCES: [1] Because of the small sample size of the HR "group", it was excluded from further analyses. Serum CXCL13 levels were higher in all pSS groups compared to healthy controls (p <0.0001), and the levels in patients with a history of NHL were higher compared to those without (p =0.0311).…”

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confidence: 99%

“…Several studies suggested reduced CVD mortality in RA in recent decades[1][2][3]. Longitudinal studies on trends in occurrence of CVD events in RA patients over time, and studies comparing trends in CVD events in RA vs general population are lacking.…”

mentioning

confidence: 99%

FRI0652 SERUM CXCL13 LEVELS ARE ASSOCIATED WITH LYMPHOMA RISK AND LYMPHOMA OCCURRENCE IN PRIMARY SJöGREN’S SYNDROME

Traianos

Locke

Lendrem

et al. 2019

Epidemiology, Risk Factors for Disease or Disease Progression

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BackgroundPrimary Sjögren’s syndrome (pSS) is an autoimmune disease characterized by an increased risk for non-Hodgkin lymphoma (NHL) development. Ectopic germinal center (GC) formation in the salivary gland (SG) epithelium of pSS patients is thought to be associated with high risk for pSS-associated NHL. The chemokine (C-X-C motif) ligand 13 (CXCL13) is a major chemoattractant for B cell migration into the GC, plays an important role in the organization of B cell follicles, and enhances BCR-triggered B cell activation. The increased B cell activation within ectopic GC may lead to excessive B cell proliferation and along with genetic abnormalities may drive the transition from the early clusters of GC B cells through oligoclonal and later monoclonal B cell expansion to lymphoma.ObjectivesTo investigate the potential of CXCL13 as a biomarker to assess NHL risk in pSS.MethodsSerum CXCL13 concentrations were quantified by ELISA in 48 healthy individuals, 165 pSS patients without NHL and 38 pSS patients with NHL from the United Kingdom Primary Sjögren’s Syndrome Registry (UKPSSR). Among these patients, follow-up serum samples from 83 and 11 patients without and with NHL respectively were available and CXCL13 measured. PSS patients without NHL were stratified into low risk (LR), moderate risk (MR) and high risk (HR) groups according to the lymphoma risk assessment tool proposed by Fragkioudaki et al, 2016. Differences in CXCL13 levels among risk groups were analyzed using the Mann-Whitney test and differences in CXCL13 levels between initial (visit 1) and follow-up (visit 2) samples were measured by repeated measures MANOVA. Associations of CXCL13 with B cell markers were determined by Pearson correlation. Logistic regression analyses were performed to model the NHL risk against CXCL13 concentrations.ResultsAt visit 1, there were 24 LR, 58 MR and 1 HR pSS patients. Because of the small sample size of the HR “group”, it was excluded from further analyses. Serum CXCL13 levels were higher in all pSS groups compared to healthy controls (p <0.0001), and the levels in patients with a history of NHL were higher compared to those without (p =0.0311). Visit 1 LR patients had significantly lower CXCL13 levels than MR patients (p =0.0015) and pSS patients with NHL (p =0.0017). Serum CXCL13 levels remained stable between visit 1 and visit 2 (mean time between visits = 4 years) for all pSS groups. CXCL13 was associated with B cell markers, including Immunoglobulin G (IgG) (p = 0.0014), B-cell activating factor (p < 0.0001), beta-2 microglobulin (p < 0.0001), combined free light chains (p < 0.0001), κ light chain (p < 0.0001), λ light chain (p = 0.0002), κ-to-λ ratio (p = 0.0013), and Anti-SSA/Ro autoantibodies (p = 0.0005). Finally, serum CXCL13 levels, age, IgM, IgA, and white blood cell count were independent predictors of NHL risk score in pSS.ConclusionOur findings have demonstrated that serum CXCL13 levels were elevated in pSS patients with NHL and MR pSS patients compared to LR pSS patients and remained stable between visit 1...

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Predicting the risk for lymphoma development in Sjogren syndrome

Abstract: Supplemental Digital Content is available in the text

Cited by 159 publications

References 48 publications

Caso clínico 01-2017. Mujer de 20 años con crecimiento de glándulas salivares y lacrimales, adenopatías, fiebre y lesión pulmonar

Caso clínico 01-2017. Mujer de 20 años con crecimiento de glándulas salivares y lacrimales, adenopatías, fiebre y lesión pulmonar

Unusual association of two cases of acute myeloblastic leukaemia and possible Sjogren’s syndrome and review of literature

FRI0652 SERUM CXCL13 LEVELS ARE ASSOCIATED WITH LYMPHOMA RISK AND LYMPHOMA OCCURRENCE IN PRIMARY SJöGREN’S SYNDROME

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