Diagnosis of Systemic Lupus Erythematosus in an Unusual Presentation: What a Primary Care Physician Should Know

Abstract

Systemic Lupus Erythematosus (SLE) is a multisystem autoimmune disease affecting millions of people worldwide. It can affect any organ systems of the body. However, all systems may not be involved initially rather than they may be affected gradually, sometimes over years. Diagnosis depends on characteristic clinical features and laboratory test results. Some features such as skin rash, joint symptoms and oral ulcers are common in SLE. But initial presentation of many patients is unusual because either they do not have these common features of the disease or the presentation mimics other illnesses. As a result, delayed diagnosis and misdiagnosis are common. Therefore, high index of initial suspicion of SLE is critical. In clinical practice, SLE should be suspected in any patient presenting with an unexplained disease process involving two or more organ systems. To make a diagnosis in an unusual presentation, thorough clinical evaluation with details history of both present and past illnesses as well as laboratory tests for SLE should be performed. Usually primary-care physicians first evaluate SLE patients; but there is no single article, where all the information on when to suspect SLE in an unusual presentation, is available in an integrated form. In this article, a list of conditions, when SLE should be suspected in an unusual presentation, has been given and some relatively common areas with diagnostic challenges of SLE have been briefly described. To prepare this manuscript, most articles have been identified through 'Pubmed' search using keywords-atypical/ unusual presentation of SLE, case reports on SLE, gastrointestinal manifestations of SLE, neuropsychiatric SLE, diagnostic challenges with SLE, etc. Selected most articles are from currently medline-indexed journals.

Systemic Lupus Erythematosus in Primary Care: An Update and Practical Messages for the General Practitioner

“…Notably, many young individuals with SLE carry psychiatric diagnoses prior to being diagnosed with SLE, which was also associated with increased pre-diagnosis healthcare use ( 60 ). Conclusively, a high index of suspicion of lupus is very important and in real-world primary care practice, SLE should be suspected in any patient who presents with unexplained manifestations involving two or more systems ( 61 ).…”

Inverse Association of Parkinson Disease With Systemic Lupus Erythematosus

“…Systemic lupus erythematosus (SLE) is a multisystemic autoimmune disease affecting any organ system of the body, and occurs predominantly in women of gestation age. 1 Neuropsychiatric symptoms affect nearly half of the patients with SLE, and the 3 most common features are seizures, psychoses, and cerebrovascular diseases. 2 , 3 Parkinson disease (PD)—an unusual manifestation of central nervous system lupus 4 —is the most common neurodegenerative disorder, and clinical signs of this disease are rigidity, bradykinesia, postural instability, and tremor.…”

Phenotype Risk Score but Not Genetic Risk Score Aids in Identifying Individuals With Systemic Lupus Erythematosus in the Electronic Health Record

“…Systemic lupus erythematosus (SLE) poses a diagnostic challenge to clinicians. Patients have diverse presentations (1), and SLE symptoms can mimic other diseases (2). SLE patients can have delays in diagnosis based on atypical or incremental disease presentation (3).…”