Organ involvement other than lupus nephritis in childhood-onset systemic lupus erythematosus

Abstract

In this review we critically analyze pulmonary, gastrointestinal and cardiac manifestations of childhood-onset systemic lupus erythematosus (cSLE). Clinical manifestations of these organ systems may be the initial manifestation of cSLE; frequently occur with very active cSLE; and are potential life-threatening manifestations often presenting to the emergency department and requiring admission to the intensive care unit. Early recognition and treatment of the pulmonary, gastrointestinal and cardiac manifestations of cSLE will result in improved prognosis and better outcomes. Lupus (2016) 25, 857-863.

Juvenile systemic lupus erythematosus in Turkey: demographic, clinical and laboratory features with disease activity and outcome

“…19,21 Kidney and CNS involvement, which are responsible for the majority of the morbidities and mortalities from SLE, are more frequent and severe in children. 1,[5][6][7]33 Particularly, Asian and African American children with SLE have a more severe and active disease course with increased renal and CNS disease compared to their Caucasian counterparts. 1,4,28 In our study, the frequency of kidney involvement and biopsy-proven overt nephritis was somewhat less than in previous jSLE studies.…”

Subclinical Pulmonary Hypertension in Childhood Systemic Lupus Erythematosus Associated with Minor Disease Manifestations

“…Lupus is a multisystem disease, and the most frequent cardiopulmonary manifestations reported are pericarditis and pleuritis, generally subclinical with small effusion volumes, rarely causing pericardial tamponade and respiratory insufficiency [3,4]. Of note, serositis occurred in more than a third of patients with PH in the context of a high disease activity score (SLEDAI-2K).…”

“…Patients were divided into two groups with similar disease duration: cSLE patients with PH and cSLE patients without PH. Reticuloendothelial involvement included lymphadenopathy (peripheral lymph node enlargement [1.0 cm), hepatomegaly [based on physical examination with liver edge C2 cm below the right costal margin or imaging (ultrasound or computer tomography)] and splenomegaly [based on physical examination with palpable spleen or imaging (ultrasound or computer tomography)] [3]. Neuropsychiatric lupus included 19 syndromes according to ACR classification criteria [14].…”

“…Systemic lupus erythematosus (SLE) is a rare chronic autoimmune illness with variable disease manifestations and outcomes, and may affect the lungs and pulmonary vessels [1][2][3][4]. Pulmonary involvement has been reported in 7-31% childhood-onset systemic lupus erythematosus (cSLE) patients [2][3][4], mainly pleural effusions, pleuritis and pneumonitis [2,5,6].…”

“…Of note, pulmonary hypertension (PH) is a severe lung manifestation and has been described as case reports and case series in cSLE populations [3,[7][8][9]. However, to our knowledge there is no study evaluating PH in a large multicenter cohort of cSLE patients.…”