New Review Outlines Juvenile-onset Systemic Lupus Erythematosus Disease Management

A clinical review entitled “Mucocutaneous manifestations in juvenile-onset systemic lupus erythematosus: a review of literature” was recently published in the journal Pediatric Rheumatology.

Juvenile-onset systemic lupus erythematosus (JSLE) is a common systemic autoimmune connective tissue condition that affects children. Estimations of JSLE are of 15-20% of all SLE cases, while overall prevalence of the condition is pegged at 0.3 to 8.8 cases per 100,000 children and adolescent per year.

Symptoms of JSLE include mucocutaneous manisfestations such as malar rash; discoid rash; photosensitivity and oral ulcers. Others symptoms include generalized lupus rash, nonscarring alopecia, livedo reticularis and Raynaud’s phenomenon. Although the prevalence of mucocutaneous manifestations in pediatric and adult patients are quite similar, some lesions are clearly less common in children; such as subacute cutaneous lesions, a discoid rash and livedo reticularis.

Since these symptoms usually occur in early stages of the disease, early symptom identification such as mucocutaneous lesions is crucial for early diagnosis and to improve clinical outcomes.

Michael Beresford from the Institute of Translational Medicine at the University of Liverpool, Liverpool in the United Kingdom and colleagues, conducted a literature review in order to systematise the key features that clinicians should consider when observing and treating patients with symptoms of JSLE.

Clinical assessment is usually sufficient to make a diagnosis of mucocutaneous lupus lesions. However, skin histopathology can be used but this needs to be carefully considered in children because of scarring after this procedure. Laboratory investigations in JSLE based on the American College of Rheumatology (ACR) criteria can also guide and support the diagnosis of the affected lesions in patients diagnosed with JSLE.

In terms of disease management, treatment is tailored according to the disease severity and organ involvement. When symptoms are limited to cutaneous stage, topical corticosteroids may be helpful. In more extensive disease or where there is systemic involvement, short courses of systemic corticosteroids with concurrent use of hydroxychloroquine and/or immunosuppressive therapies are indicated.

The researchers indicate that since patients with JSLE usually present mucocutaneous manifestations, and it is important to recognize these lesions in order to make an appropriate diagnosis.

It is crucial to assess and regularly monitor the systematic disease including mucocutaneous lesions with SLC needs in children, as it may take a long time before these patients meet the diagnostic criteria for mucocutaneous lupus.

The researchers strongly encourage the use of sun protection to prevent worsening of the symptoms and exacerbation of SLE.

The researchers concluded that it is common to observe lupus with skin disease in adults. However, in children, lesions are usually associated with systemic disease and require treatment with systemic immunosuppressive drugs in order to achieve adequate disease control.