Rare clinical manifestations in systemic lupus erythematosus: a review on frequency and clinical presentation.
Author
Tani, ChiaraElefante, Elena
Arnaud, Laurent
Barreira, Sofia C
Bulina, Inita
Cavagna, Lorenzo
Costedoat-Chalumeau, Nathalie
Doria, Andrea
Fonseca, João Eurico
Franceschini, Franco
Fredi, Micaela
Iaccarino, Luca
Limper, Maarten
Majnik, Judit
Nagy, Gyorgy
Pamfil, Cristina
Rednic, Simona
Reynolds, John A
Tektonidou, Maria G
Troldborg, Anne
Zanframundo, Giovanni
Mosca, Marta
Affiliation
Azienda Ospedaliero Universitaria Pisana; Hôpitaux Universitaires de Strasbourg; Hospital de Santa Maria; Sandwell and West Birmingham NHS Trust; et al.Publication date
2022-04-29Subject
Rheumatology
Metadata
Show full item recordAbstract
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.Citation
Tani C, Elefante E, Arnaud L, Barreira SC, Bulina I, Cavagna L, Costedoat-Chalumeau N, Doria A, Fonseca JE, Franceschini F, Fredi M, Iaccarino L, Limper M, Majnik J, Nagy G, Pamfil C, Rednic S, Reynolds JA, Tektonidou MG, Troldborg A, Zanframundo G, Mosca M. Rare clinical manifestations in systemic lupus erythematosus: a review on frequency and clinical presentation. Clin Exp Rheumatol. 2022 May;40 Suppl 134(5):93-102. doi: 10.55563/clinexprheumatol/jrz47c.Type
ArticlePMID
35485422ae974a485f413a2113503eed53cd6c53
10.55563/clinexprheumatol/jrz47c