Egyptian Journal of Pediatric Allergy and Immunology (The)

Infective endocarditis in a previously healthy adolescent masquerading as lupus in a COVID-19 era

2024-04-21T17:45:20+00:00

Background: Multisystem inflammatory syndrome of children (MIS-C) has emerged as a serious systemic inflammatory disorder complicating COVID-19 infection in children. Arterial and venous thrombosis have been reported complicating COVID-19 infection but not infective endocarditis (IE).

Case presentation: Herein, we describe a 12-year-old girl initially presented with picture of MIS-C with echocardiography (ECHO) revealing only pericardial effusion, for which she has received single dose of pulsed methylprednisolone with no improvement, her blood picture was significant for anemia and thrombocytopenia, with increased acute phase reactants (APRs) including serum ferritin and IL-6. She was found to have as well oral ulcers, polyarthritis, vascular purpura, and exaggerated deep tendon reflexes. She had history of low-grade fever, gastrointestinal illness, together with myalgia and easily fatigability during the last month before presentation, symptoms suggestive of viral infection most probably SARS-CoV-2 infection with positive COVID-19 IgG. The girl was considered as probable systemic lupus erythematosus (SLE) in face of negative immunological evaluation for SLE except for moderately positive anti-cardiolipin IgM. Initially, the girl has responded to intravenous steroids but ten days later, she came back toxic with marked increase in APRs and ECHO revealed left atrial mural highly mobile floating mass, blood culture was positive for staphylococcus coagulase negative organism, steroids were gradually withdrawn, and complete resolution of the IE was achieved after six weeks of parenteral antibiotics.

Conclusion: COVID-19 infection can result in subclinical cardiac affection making the heart vulnerable to colonization with even mild bacteremia. Although autoimmune features of IE are uncommon but can confuse the presentation especially when the vegetations could not initially be detected by ECHO.

Assessment of serum and cutaneous JAK3 in juvenile scleroderma

2024-04-21T17:08:03+00:00

Background: Janus kinases (JAKs) family include JAK 1, 2, 3 and tyrosine kinase 2 play central role in cytokine and growth factor signaling and few studies suggest their possible contribution in the pathogenesis of scleroderma.
Objective: To evaluate serum and cutaneous expression of JAK3 in pediatric patients with juvenile scleroderma (JSD) and their association with the disease severity .
Methods: This was a pilot study that included 16 pediatric patients with JSD; they were 11 patients with juvenile systemic sclerosis (JSSc) and 5 patients with juvenile localized scleroderma (JLS). The patients were compared to 17 healthy controls. Disease severity was assessed using modified Rodnan skin score (mRSSc) and JSSc severity score (J4S) in JSSc patients while localized scleroderma damage index (LoSDI) was used in JLS patients. Serum and cutaneous expression of JAK3 were measured by enzyme linked immunosorbent assay .
Results: The median (IQR) of serum JAK3 was significantly higher among JSSc patients as compared to that of controls [430 (320-520) versus 270 (180-385), p = 0.005], while comparable values were found among JLS and controls as well as JLS and JSSc patients (p>0.05). Cutaneous expression of JAK3 was comparable between all patients and controls (p>0.05). Serum and cutaneous expression of JAK 3 did not correlate significantly with mRSSc and J4S in JSSc patients and LoSDI in JLS patients (p>0.05) .
Conclusion: JAK3 seems to contribute to the pathogenesis of JSSc and further studies are needed to establish its role and the usefulness of using JAK 3 inhibitors in these patients.

NLR pyrin domain 3 (NLRP3) protein expression in juvenile systemic lupus erythematosus and lupus nephritis

2024-04-21T17:31:16+00:00

Background: In systemic lupus erythematosus (SLE), the increased rate of apoptosis and inefficient clearance of apoptotic cells leads to activation of NLRP3 inflammasome. NLRP3 overstimulation is proved to be involved in the pathogenesis of lupus nephritis (LN). We sought to measure the expression of NLRP3 among pediatric patients with SLE and LN, in correlation to markers of activity.

Methods: A pilot, cross-sectional controlled study was conducted from Jan 2022 to Jan 2024. Fifty-six patients with confirmed SLE and active LN with or without other system activity were compared to age and sex matched 56 healthy controls as regards expression of NLRP3 protein using enzyme linked immunosorbent assay (ELISA) technique (Human NLRP3 ELISA Kit).

Results: Peripheral expression of NLRP3 was significantly higher among patients with juvenile SLE with active LN than controls (p value <0.001). Other systemic activities were present within 27/56 patients, 11 patients had cardiac manifestations and 16 patients had neurological manifestations. SLE disease activity index (SLEDAI) was 32.4 (SD 7.6) and was significantly correlated to peripheral NLRP3. LN Class, level of C3 consumption, proteinuria and kidney functions were not correlated to levels of NLRP3 protein.

Conclusion: Expression of NLRP3 inflammasome in the peripheral blood was significantly elevated in juvenile SLE with LN compared to controls, with significant correlation to SLEDAI, but not to LN Class or markers of activity. Comparison between pediatric patients with SLE with and without LN and comparing renal NLRP3 inflammasome to its peripheral expression in patients with LN are recommended.