Atypical Initial Presentations of SLE – Our Experience From A Tertiary Centre In Eastern India

Systemic Lupus Erythematosus (SLE) is the prototypical multisystem connective tissue disorder affecting persons of all age and both sexes although it is more common in women with childbearing age [1]. The disease was first described by Italian physician Rogerius in 12th century in patients presenting with malar rash, but the systemic nature of the disease was established much later in 1872 by Moric Kaposi [2]. Currently the prevalence of SLE worldwide is reported between 17 to 48 per 100000 individuals [3]. The hallmark characteristics of the disease is production of large amount of different kinds of autoantibodies detectible in blood and other body fluids. Because of its wide variety of manifestations SLE has been previously dubbed as the “great mimicker” [1]. The Systemic Lupus Collaborating Clinics (SLICC) criteria and more recently the ACR-EULAR criteria is used to make a diagnosis of SLE compiling both clinical and immunological features [4]. In terms of presenting features of SLE, the usual symptoms which we encounter in our clinical life are polyarthralgia/arthritis (58%), fever (33%), alopecia (32%), discoid rash (29%), weight loss (28%), buccal ulcer (22%), fatigue (18%), pleuritic chest pain (17%), malar rash, headache, seizures, recurrent abortion, photosensitivity, cognitive impairment etc [3]. However, time and again SLE presents with certain unusual symptoms which baffles us making it difficult and time-consuming to diagnose. There are sporadic case reports of some such presentations in the various public domains.