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Rare clinical manifestations in systemic lupus erythematosus: a review on frequency and clinical presentation

1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22

 

  1. Rheumatology Unit, Azienda Ospedaliero Universitaria Pisana, Pisa, Italy. chiaratani78@gmail.com
  2. Rheumatology Unit, Azienda Ospedaliero Universitaria Pisana, Pisa, Italy.
  3. Department of Rheumatology, Hôpitaux Universitaires de Strasbourg, INSERM UMR-S 1109, Centre National de Référence des Maladies Auto-immunes Systémiques Rares (RESO), Strasbourg, France.
  4. Rheumatology Department, Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa Norte, Lisbon Academic Medical Centre and European Reference Network on Rare Connective Tissue and Musculoskeletal Diseases Network (ERN-ReCONNET), Lisbon, and Rheumatology Research Unit, Instituto de Medicina Molecular João Lobo Antunes, Faculdade de Medicina, Universidade de Lisboa, Portugal.
  5. Reimatoloģijas centra vadītāja VSIA P. Stradiņa KUS IMK Reimatoloģijas centrs, LPRB (Latvijas pieaugušo Reimatoloģijas biedrība) valdes locekle, ERN ReCONNET Affiliated Partner representative of Latvia adults rheumatologists, Pauls Stradins Clinical University hospital, Internal Medicine Clinic, Centre of Rheumatology, Riga, Latvia.
  6. Rheumatology Division, University and IRCCS Policlinico S. Matteo Foundation, Pavia, Italy.
  7. Service de Médecine Interne, Centre de Référence Maladies Autoimmunes et Systémiques Rares Île de France, APHP, Hôpital Cochin, Paris, and Université de Paris, Centre de Recherche Épidémiologie et Biostatistiques de Sorbonne Paris Cité, France.
  8. Dipartimento di Medicina-DIMED, Azienda Ospedaliera/Università di Padova, Italy.
  9. Rheumatology Department, Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa Norte, Lisbon Academic Medical Centre and European Reference Network on Rare Connective Tissue and Musculoskeletal Diseases Network (ERN-ReCONNET), Lisbon, and Rheumatology Research Unit, Instituto de Medicina Molecular João Lobo Antunes, Faculdade de Medicina, Universidade de Lisboa, Portugal.
  10. Rheumatology and Clinical Immunology Unit, ASST Spedali Civili, Brescia, and Department of Clinical and Experimental Sciences, University of Brescia, Italy.
  11. Rheumatology and Clinical Immunology Unit, ASST Spedali Civili, Brescia, and Department of Clinical and Experimental Sciences, University of Brescia, Italy.
  12. Dipartimento di Medicina-DIMED, Azienda Ospedaliera/Università di Padova, Italy.
  13. Department of Rheumatology and Clinical Immunology, Division of Internal Medicine and Dermatology, University Medical Centre Utrecht, Utrecht University, The Netherlands.
  14. Semmelweis University, Department of Rheumatology and Clinical Immunology, Hospital of Hospitaller Brothers of St. John of God, Rheumatology and Clinical Immunology, Department of Genetics, Cell- and Immunobiology Semmelweis University, Budapest, Hungary.
  15. Semmelweis University, Department of Rheumatology and Clinical Immunology, Hospital of Hospitaller Brothers of St. John of God, Rheumatology and Clinical Immunology, Department of Genetics, Cell- and Immunobiology Semmelweis University, Budapest, Hungary.
  16. Department of Rheumatology, Iuliu Hatieganu University of Medicine and Pharmacy, County Emergency Clinical Hospital Cluj, Cluj-Napoca, Romania.
  17. Department of Rheumatology, Iuliu Hatieganu University of Medicine and Pharmacy, County Emergency Clinical Hospital Cluj, Cluj-Napoca, Romania.
  18. Rheumatology Research Group, Institute of Inflammation and Ageing, College of Medical and Dental Sciences, University of Birmingham, and Rheumatology Department, City Hospital, Sandwell and West Birmingham NHS Trust, Birmingham, UK.
  19. Rheumatology Unit, First Department of Propaedeutic Internal Medicine, Joint Rheumatology Academic Program, School of Medicine, National and Kapodistrian University of Athens, Greece.
  20. Department of Rheumatology, Aarhus University Hospital, and Department of Biomedicine, Aarhus University, Denmark.
  21. Rheumatology Division, University and IRCCS Policlinico S. Matteo Foundation, Pavia, Italy.
  22. Rheumatology Unit, Azienda Ospedaliero Universitaria Pisana, and Department of Clinical and Experimental Medicine, University of Pisa, Italy.

CER15443
2022 Vol.40, N°5 ,Suppl.134
PI 0093, PF 0102
Reviews

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PMID: 35485422 [PubMed]

Received: 26/12/2021
Accepted : 17/03/2022
In Press: 29/04/2022
Published: 18/05/2022

Abstract

OBJECTIVES:
The purpose of this study was to review the frequency and clinical presentation of the rarest clinical manifestations of systemic lupus erythematosus (SLE).
METHODS:
A list of 6 rare SLE manifestations were defined: gastrointestinal, liver, pulmonary, cardiac, ocular and neurological manifestations. Each topic was assigned to a pair of authors to perform a literature search and article review.
RESULTS:
In total, 149 articles were included in the literature review: 37 for gastrointestinal manifestations, 6 for liver manifestations, 27 for pulmonary manifestations, 50 for cardiac manifestations, 16 for ocular manifestations, 13 for neurological manifestations. Gastrointestinal disorders included several clinical presentations with variable frequency (from 0.5% to 10.7% of the cases); liver involvement included lupus-related hepatitis (9.3%) and autoimmune hepatitis (2.3%). The rarest pulmonary manifestations identified were shrinking lung syndrome, described in 1.5% of patients, while interstitial lung disease and lupus pneumonia were reported in 4% and 3% of patients respectively. Myocarditis and pulmonary hypertension were also rarely described in SLE patients although ranging from 0.4-16% and 1-14% respectively, depending on the methodology used for its identification. Ocular manifestations in SLE included some rare manifestations (reported in less than 5% of patients) and lupus retinopathy that is described in 1.2-28.8% of patients depending on methods of ascertainment. Aseptic meningitis and chorea were also confirmed as very rare manifestations being reported in less than 1% and in 0.3-2.4% of cases respectively.
CONCLUSIONS:
The results of this literature review provide the basis for a better understanding of some less-known manifestations of SLE and for stressing the need for a higher awareness in diagnostic and therapeutic protocols regarding these rare disease aspects.

DOI: https://doi.org/10.55563/clinexprheumatol/jrz47c

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