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Joint Bone Spine. 2019 Mar;86(2):165-171. doi: 10.1016/j.jbspin.2018.10.007. Epub 2018 Oct 26.

Rare diseases that mimic Systemic Lupus Erythematosus (Lupus mimickers).

Author information

1
Service de dermatologie et d'allergologie, hôpital Tenon, AP-HP, 75020, Paris, France.
2
Immunoconcept, CNRS-UMR 5164, université de Bordeaux, 146, rue Léo-Saignat, 33076, Bordeaux, France; Centre hospitalier universitaire de Bordeaux, FHU ACRONIM, place Amélie-Raba-Léon, 33076, Bordeaux, France; Centre national de référence des maladies autoimmunes et systémiques rares Est Sud-Ouest (RESO)-LUPUS, 67000 Strasbourg, France.
3
Centre national de référence des maladies autoimmunes et systémiques rares Est Sud-Ouest (RESO)-LUPUS, 67000 Strasbourg, France; Service d'immunologie clinique nouvel hôpital civil, 1, place de l'hôpital 67091 Strasbourg cedex, France.
4
Service de nephrologie, rhumatologie et dermatologie pédiatriques, hôpital Femme Mère-Enfant, hospices civils de Lyon, Lyon, France; Université de Lyon, 59, boulevard Pinel, 69677 Bron cedex, France; Inserm U1111, 69007 Lyon, France; Filière des maladies autoimmunes et autoinflammatoires rares (FAI2R), 69677 Lyon, France.
5
Centre national de référence des maladies autoimmunes et systémiques rares Est Sud-Ouest (RESO)-LUPUS, 67000 Strasbourg, France; Service de rhumatologie, hôpitaux universitaires de Strasbourg, 67098 Strasbourg, France; Université de Strasbourg, Inserm UMR-S 1109, 67000 Strasbourg, France. Electronic address: Laurent.arnaud@chru-strasbourg.fr.

Abstract

Several conditions have clinical and laboratory features that can mimic those present in Systemic Lupus Erythematosus (SLE). Some of these "SLE mimickers" are very common, such as rosacea which can be mistaken for the butterfly rash, while others such as Kikuchi disease, type-1 interferonopathies, Castleman's disease, prolidase deficiency, angioimmunoblastic T-cell lymphoma, Evans' syndrome in the context of primary immune deficiencies and the autoimmune lymphoproliferative syndrome are exceptionally uncommon. A proper diagnosis of SLE must therefore be based upon a complete medical history as well as on the adequate constellation of clinical or laboratory findings. While there is no single test that determines whether a patient has lupus or not, the search for auto-antibodies towards nuclear antigens is a key step in the diagnosis strategy, keeping in mind that ANAs are not specific for SLE. In case of persistent doubt, patients should be referred to reference centers with experience in the management of the disease.

KEYWORDS:

Differential/diagnosis; Lupus erythematosus; Review; Systemic/diagnosis

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