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

Abstract

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 couple 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 lupusrelated 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. Conclusion. 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.

Systemic Lupus Erythematosus

“…Gastrointestinal manifestations of SLE include protein-losing enteropathy, hepatitis, pancreatitis, and intestinal pseudo-obstruction, which presents with clinical features of intestinal obstruction without an identifiable obstructive lesion . Each of these conditions affects less than 10% of patients with SLE.…”

Chorea as an initial and solitary manifestation of systemic lupus erythematosus with antiphospholipid syndrome in an elderly man

“…The pathogenesis of SLE or APS-associated chorea could be related to a vascular, or immune-mediated neuronal, and glial injury resulting in dysfunctional basal ganglia circuitry. It is a combination of either vascular injury induced by circulating immune complex, occlusive vasculopathy because of endothelial cell activation induced by cytokines and complement activation, or macrovascular and microvascular thrombosis induced by antiphospholipid (aPL) antibodies 6 7. The prevalence of aPL antibodies especially anti- B2GPI antibodies are higher in SLE patients with neuropsychiatric disease.…”

Brief Review About Neurological, Hematological, Gastrointestinal, Cardiovascular and Pulmonar Manifestations of Systemic Erythematosus Lupus

“…Now, it is clear that cardiovascular pathology due to systemic lupus erythematosus is performed after ruling out pleuritis, pericarditis, pulmonary emboli, pneumonia, interstitial lung pathology and even gastroesophageal reflux. In general, prevention and treatment of cardiovascular disease is based on not smoking, regular exercise, optimization of lipid levels, controlling blood pressure, with prophylactic use of aspirin and minimizing the use of glucocorticoids; hydroxychloroquine, which is widely used in lupus, has been shown to have an additional antithrombotic and anti-atherogenic benefit (14) (15).…”