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…
SjÖgren Syndrome The syndrome of chronic inflammation of the exocrine glands, principally the salivary and lacrimal glands, was first described by Henrik Sjögren, a Swedish ophthalmologist, who published in 1930 the first complete description of a disorder he named keratoconjunctivitis…
Scleroderma refers to hard skin that results from an excessive deposition of collagen. In the localized form (localized scleroderma [LS], also called morphea ), inflammation in the skin and subcutaneous tissues triggers the process of fibrosis. LS and the systemic…
Introduction The scleroderma disorders in children encompass both systemic sclerosis (SSc) and localized scleroderma (LS), also termed morphea . Juvenile systemic sclerosis (JSSc) is much less common than juvenile localized scleroderma (JLS) and comprises less than 10% of all SSc…
Idiopathic inflammatory myopathies (IIM) are a heterogenous group of diseases characterized by muscle weakness (which is often more pronounced proximally) and inflammatory changes in skeletal muscle. IIM comprise different subtypes: dermatomyositis, polymyositis, inclusion body myositis, and amyopathic dermatomyositis. Juvenile dermatomyositis…
Neonatal lupus erythematosus (NLE) is a disease of the developing fetus and neonate defined by characteristic clinical features in the presence of specific maternal autoantibodies. The first reported case of congenital heart block (CHB) associated with maternal autoimmune disease (i.e.,…