Kawasaki Disease
Historical Background Kawasaki disease (KD) is one of the most common vasculitides of childhood. It has the potential to cause severe complications, significant morbidity, and even mortality. Expeditious treatment can…
Antineutrophil Cytoplasmic Antibody Associated Vasculitis
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 of…
Leukocytoclastic Vasculitis: Henoch–Schönlein Purpura and Hypersensitivity Vasculitis
Necrotizing vasculitis that affects small blood vessels (especially the postcapillary venules, capillaries, and arterioles) and is often caused by immune complex deposition may show leukocytoclastic vasculitis on histology ( Fig….
Other Vasculitis
Cogan Syndrome Cogan syndrome was first described in 1945 by Dr. David Cogan, an ophthalmologist, who described four patients who developed near simultaneously interstitial keratitis and vestibular-auditory symptoms, which led…
Pediatric Sarcoidosis
Pediatric sarcoidosis comprises a spectrum of childhood granulomatous inflammatory conditions with the hallmark being the presence of noncaseating epithelioid giant cell granulomas in a variety of tissues and organ systems….
Reactive Arthritis
The reactive arthritides constitute a group of diverse inflammatory arthropathies in which the joint and extraarticular manifestations are caused by a preceding extraarticular infection with specific bacteria. This chapter reviews…
Pain Amplification Syndromes
Pediatric rheumatologists encounter children with acute and chronic pain for which an overt primary cause for the pain cannot be found. In these disorders the pain seems disproportional or amplified….
Raynaud Phenomenon and Vasomotor Syndromes
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….
Lyme Disease
Lyme arthritis was first described in 1977 by Steere and colleagues in a cluster of children thought to have juvenile rheumatoid arthritis. They lived in and around Old Lyme, Connecticut….
Polyarteritis Nodosa
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…
