Repeated febrile episodes lasting for a few days to a few weeks are common in young children. Infection is the most common etiological factor. However, after infectious causes and malignancy have been excluded, rheumatic illnesses enter the differential diagnosis. Unexplained…
Introduction and Historical Review In 1937 the Turkish dermatologist Hulusi Behçet described the syndrome that bears his name, comprising the clinical triad of aphthous stomatitis, genital ulceration, and uveitis. Subsequently Adamantiades defined the thrombophlebitis and Matteson identified even earlier reports…
Takayasu Arteritis In 1908 the Japanese ophthalmologist Takayasu and his colleagues described the association of retinal vessel abnormalities with absent wrist pulses. Takayasu arteritis (TA) is a rare, chronic, relapsing granulomatous large-vessel vasculitis affecting the aorta, its major branches, and…
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of vasculitides characterized by small- to medium-sized blood vessel inflammation, clinically overlapping features, and the presence of ANCA. This group comprises granulomatosis with polyangiitis (GPA), formerly Wegener granulomatosis; microscopic polyangiitis (MPA);…
Historical Background Kawasaki disease (KD) is one of the most common vasculitides of childhood. This vasculitis bears the name Kawasaki disease because of the highly detailed description of this illness in 50 children by Tomisaku Kawasaki in 1967. Scattered case…
Introduction Polyarteritis nodosa (PAN) was first described by Kussmaul and Maier in 1866. The original and subsequent descriptions identified the pathological features of necrotizing arteritis with nodules along the walls of medium and small muscular arteries, leading to thrombosis or…
Necrotizing vasculitis that affects small blood vessels (especially the postcapillary venules, capillaries, and arterioles) is often caused by immune complex deposition and may show leukocytoclastic vasculitis on histology ( Fig. 33.1 and Box 33.1 ). , The term leukocytoclasis refers…
The term vasculitis indicates the presence of inflammation in a blood vessel wall. The inflammatory infiltrate may be one that is predominantly neutrophilic, eosinophilic, or mononuclear. Perivasculitis describes inflammation around the blood vessel wall but without mural involvement. Vasculopathy, a…
Acknowledgements The authors wish to express their gratitude to Drs. Adam Kirton and Susanne Benseler who authored the previously published version of this chapter. Approach to Inflammatory Brain Disorders Inflammatory disorders of the central nervous system encompass an array of…
Introduction Episodic color changes of the hands and feet in response to cold or stress, known as Raynaud phenomenon (RP), are a frequent complaint among patients presenting to pediatric rheumatology clinics. The first description of vasomotor instability triggered by cold…