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

Chiara Tani, Elena Elefante, Laurant Arnaud, Sofia Barreira, Inita Bulina, Lorenzo Cavagna, Nathalie Costedoat-Chalumeau, Andrea Doria, Joao Fonseca, Franco Franceschini, Micaela Fredi, Luca Iaccarino, Maarten Limper, Judit Majnik, Gyorgy Nagy, Christina Pamfil, Simona Rednic, John Reynolds, Maria Tektonidou, Anne TroldborgGiovanni Zanframundo, Marta Mosca

Research output: Contribution to journalReview articlepeer-review


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.
Original languageEnglish
Pages (from-to)S93-S102
Number of pages10
JournalClinical and Experimental Rheumatology
Issue number5, Suppl 134
Publication statusPublished - 18 May 2022

Bibliographical note

Funding Information:
Funding and disclaimer: page S99. Competing interests: L. Arnaud has acted as a consultant for Alexion, Amgen, Astra-Zeneca, Abbvie, Biogen, BMS, Boehringer-Ingelheim, Cemka, GSK, Grifols, Janssen-Cilag, Kezar, LFB, Lilly, Medac, Novartis, Oséus, Pfizer, Roche-Chugai, Semeia, UCB. I. Bulina has received honoraria from Abbvie, Boehringer-Ingelheim, Janssen and Pfizer. N. Costedoat-Chalumeau has received grants from UCB for a clinical research study. The other authors have declared no competing interests.

Publisher Copyright:
© Copyright Clinical and Experimental Rheumatology 2022.


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