Systemic phenotype related to primary Sjögren's syndrome in 279 patients carrying isolated anti-La/SSB antibodies

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OBJECTIVES: To evaluate the systemic phenotype associated with the presence of isolated anti-La/SSB antibodies in a large international registry of patients with primary Sjögren's syndrome (pSS) fulfilling the 2002 classification criteria.

METHODS: The Big Data Sjögren Project Consortium is an international, multicentre registry created in 2014. Baseline clinical information from leading centres on clinical research in SS of the 5 continents was collected. Combination patterns of anti-Ro/SSA-La/SSB antibodies at the time of diagnosis defined the following four immunological phenotypes: double positive (combined Ro/SSA and La/SSB,) isolated anti-Ro/SSA, isolated anti-La/SSB, and immunonegative.

RESULTS: The cohort included 12,084 patients (11,293 females, mean 52.4 years) with recorded ESSDAI scores available. Among them, 279 (2.3%) had isolated anti-La/SSB antibodies. The mean total ESSDAI score at diagnosis of patients with pSS carrying isolated anti-La/SSB was 6.0, and 80.4% of patients had systemic activity (global ESSDAI score ≥1) at diagnosis. The domains with the highest frequency of active patients were the biological (42.8%), glandular (36.8%) and articular (31.2%) domains. Patients with isolated anti-La/SSB showed a higher frequency of active patients in all ESSDAI domains but two (articular and peripheral nerve) in comparison with immune-negative patients, and even a higher absolute frequency in six clinical ESSDAI domains in comparison with patients with isolated anti-Ro/SSA. In addition, patients with isolated anti-La/SSB showed a higher frequency of active patients in two ESSDAI domains (pulmonary and glandular) with respect to the most active immunological subset (double-positive antibodies). Meanwhile, systemic activity detected in patients with isolated anti-La/SSB was overwhelmingly low. Even in ESSDAI domains where patients with isolated anti-La/SSB had the highest frequencies of systemic activity (lymphadenopathy and muscular), the percentage of patients with moderate or high activity was lower in comparison with the combined Ro/SSA and La/SSB group.

CONCLUSIONS: Patients carrying isolated La/SSB antibodies represent a very small subset of patients with a systemic SS phenotype characterised by a significant frequency of active patients in most clinical ESSDAI domains but with a relative low frequency of the highest severe organ-specific involvements. Primary SS still remains the best clinical diagnosis for this subset of patients.


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Externa organisationer
  • University of Debrecen
  • Skåne University Hospital
  • Dipartimento di Ingegneria Chimica, Sapienza – Università di Roma
  • Hanze University of Applied Sciences
  • Instituto Nacional de Ciencias Médicas y Nutrición
  • Hacettepe University Department of Clinical Microbiology, Ankara
  • McMaster University Medical Centre
  • Wrocław Medical University
  • University of Perugia
  • University of Adelaide
  • Hospital das Clínicas da Universidade de São Paulo
  • Uppsala University, Faculty of Theology
  • Nagasaki University
  • Federal University of São Paulo
  • Instituto Modelo de Cardiología Privado SRL
  • University Hospital Complex Of Vigo
  • Hospital Rey Juan Carlos de Móstoles
  • Hospital Joan XXIII
  • University Hospital Cabueñes
  • Hospital de Clínicas "José de San Martín"
  • University of Newcastle upon Tyne
  • Peking Union Medical College
  • Anhui Provincial Hospital
  • University of Pisa
  • INSERM U1184
  • University of Strasbourg
  • University Medical Center Utrecht
  • Christian Medical College & Hospital
  • University Hospital 'Santa Maria della Misericordia'
  • Catholic University of Korea
  • Karolinska University Hospital
  • Vall d'Hebron University Hospital
  • Kanazawa University Hospital
  • University College London Hospital
  • Federal University of Espírito Santo
  • Technical University of Munich
  • University of L'Aquila
  • University Hospital of Brest
  • University of Messina
  • Cairo University
  • University of Barcelona
  • Royal Observatory of Madrid
  • University of Groningen
  • Hospital Clínic of Barcelona
  • Polytechnic University of Catalonia
  • Autonomous University of Barcelona

Ämnesklassifikation (UKÄ) – OBLIGATORISK

  • Reumatologi och inflammation
Sidor (från-till)85-94
Antal sidor10
TidskriftClinical and Experimental Rheumatology
Volym38 Suppl 126
Utgåva nummer4
StatusPublished - 2020 okt 24
Peer review utfördJa