Is There an Association Between Initial Clinical Manifestations and the Development of Macrophage Activation Syndrome in Patients with Systemic Juvenile Idiopathic Arthritis?

Objective: Systemic juvenile idiopathic arthritis is deemed as a subtype of the disease complex known as juvenile idiopathic arthritis but differs in the point of its clinical manifestations and pathophysiological features. Besides, macrophage activation syndrome is a potentially fatal complication of systemic juvenile idiopathic arthritis requiring timely management. The study aims to evaluate the presence and recurrence of macrophage activation syndrome according to the initial symptoms and pattern of different phenotypes in systemic juvenile idiopathic arthritis to reveal the comprehensive association of these interrelated disorders

Autoimmune and autoinflammatory diseases with mucocutaneous manifestations: A pediatric rheumatology perspective

The presence of mucocutaneous manifestations has clinical significance, as it may be a part of the initial presentation or activation stage of both autoimmune and autoinflammatory rheumatic diseases. The cutaneous signs may display a particular morphological and topographic distribution according to taxonomy, whereas heterogeneity is likely observed among the individuals. The review aims to cluster and systematically approach the mucocutaneous manifestations met in autoimmune and autoinflammatory rheumatic diseases of childhood. The search strategy involved a comprehensive inquiry on Web of Science, PubMed, MEDLINE, and Embase databases using relevant search terms such as "dermatologic, cutaneous, mucocutaneous, skin, rash" for each disease and category. The awareness of the distinctive mucocutaneous manifestations and their correlation with rheumatic diseases provides a convenient definition, well-timed control of the underlying condition, and prevention of cosmetic issues. In the management of rheumatic diseases, planning the pertinent differential diagnosis and determining the requirement of histopathological assessment are essential with a multidisciplinary approach to rheumatology, dermatology, and allergy-immunology specialties

Thrombotic thrombocytopenic purpura as a rare cause of anemia with thrombocytopenia in childhood: report of 2 cases

Thrombotic thrombocytopenic purpura (TTP) is a rare multisystem disorder characterized by single or recurrent episodes of thrombocytopenia, microangiopathic hemolytic anemia and widespread microvascular thrombosis, which causes significant morbidity and mortality unless promptly recognized and treated. The underlying pathogenesis is a defect in von Willebrand factor (vWF) cleaving protease, called "A Disintegrin and Metalloproteinase with Thrombospondin Type 1 Repeats 13 (ADAMTS-13)". There are 2 forms: congenital TTP (ADAMTS-13 gene mutations) and acquired TTP (autoantibodies and ADAMTS-13 deficiency). We presented two patients who initially presented with thrombotic microangiopathy and were later diagnosed with TTP upon demonstration of the deficiency in ADAMTS-13 activity

Pediatric Behcet's disease - clinical aspects and current concepts

Behcet's Disease was first described by a Turkish dermatologist, Hulusi Behcet, in 1937 as a triple symptom complex; aphthous stomatitis, genital ulcers, and uveitis. Today, in light of current trials and experiments, we know that the disease may have a wider involvement with a multisystemic recurrent course, causing significant morbidity and mortality. However, there are still unanswered questions, particularly about Pediatric Behcet's Disease. Although several immunological and genetic associations have been demonstrated, the real etiologic mechanism of the disease is unclear. The diagnosis is difficult due to its rarity in childhood, the lack of validation of the diagnostic criteria obtained from adult studies, and the inadequacy of large case-controlled studies. Also, the management is challenging and controversial due to the various geographic distribution of clinical spectrum. New therapeutic options under development in light of pathogenetic hypothesis seem to be promising

SHEAR WAVE ELASTOGRAPHY OF SALIVARY AND THYROID GLANDS IN PEDIATRIC PATIENTS WITH FMF AND AMYLOIDOSIS

Objective: The aim of this study is to investigate the effects of recurrent inflammation and amyloid deposition on salivary and thyroid gland elasticity in pediatric patients with Familial Mediterranean Fever (FMF) using shear wave ultrasound elastography