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Vasculitis Vasculitis is inflammation of vessel walls

The Spectrum of Vasculitides in the Pediatric Rheumatology Clinic “Common” vasculotides: HSP Kawasaki disease Cutaneous vasculitis: LCV cutaneous PAN microscopic polyangiitis spider bite

The Spectrum of Vasculitides in the Pediatric Rheumatology Clinic Systemic diseases: JDM Behcet PSS localized scleroderma Odd cases: Wegener, sarcoidosis, polymyositis, CINCA, Tulosa Hunt, Takayasu, urticarial vasculitis, isolated CNS vasc. Isolated lung vasc…….

vasculitides indistinguishable clinical presentations different prognoses and treatments substantial overlap among different vasculitides

Constitutional signs and symptoms fever myalgias malaise a "flu-like" syndrome early in the course arthralgias - migratory - both small and large joints synovitis peripheral neuropathy, especially mononeuritis multiplex

Visceral involvement intussusception and perforation pancreatitis upper respiratory tract disease lower respiratory tract disease CNS disease hepatitis glomerulonephritis

Henoch–Schonlein purpura Henoch–Schnlein purpura is a specific vasculitic syndrome which is due to IgA immune complexes and IgA deposition within the vessels and the kidney.

HSP The full spectrum includes palpable purpura, nephritis, arthritis and gastrointestinal involvement.

HSP The onset of this disorder is often acute and frequently resolution is rapid and complete. HSP is more frequent in boys. There are patients who will have a continued nephropathy with resultant nephrotic syndrome or, on rare occasions, renal failure.

HSP Of 30 children restudied from 1.5 to 15 years after onset of HSP, only five had renal abnormalities, and only one had renal failure. In adults with HSP, the prognosis is perhaps more guarded with more frequent renal abnormalities.

Skin The skin involvement is one of the defining characteristics of HSP.  The rash may begin as a macular erythema, urticarial lesions, but may progress to purpura rapidly. The lower extremities and buttocks are the most common sites for the rash.

DD Other vasculitic syndromes, infections such as meningococcemia, subacute bacterial endocarditis, and Rocky Mountain Spotted fever must also be considered in the differential diagnosis.

HSP Most often the purpura are part of the presenting manifestations, but in some cases arthritis or abdominal symptoms may occur for up to several weeks prior to the onset of rash.

HSP This disorder is a systemic process and thus the joints, gastrointestinal tract, and kidneys are frequently involved. Furthermore, patients with IgA nephropathy may also be part of the spectrum of this disease since they also demonstrate IgA circulating immune complexes

Joints The joints are involved in 60–90% of patients with HSP. The involvement is symmetrical and most commonly involves the ankles, knees, metacarpophalangeal and metatarsophalangeal joints. It is not clear whether the joint pain is due to synovitis or to periarticular edema.

GI Gastrointestinal involvement is the most serious acute manifestation. The gastrointestinal tract findings are due to vasculitis within the bowel wall with resultant edema. This can cause the colicky pain and hemorrhage resulting in gastrointestinal bleeding. Severe involvement can result in perforation.

Kidney Nephritis is usually asymptomatic, but gross hematuria can occur on rare occasions. Hematuria and proteinuria are the usual findings and occur in 30–90% of patients. Both the bowel and kidney have been demonstrated to contain IgA deposits within vessels.

Etiology The cause of HSP is unknown. While some cases have been preceded by a streptococcal infection, a wide array of other infectious agents have been implicated. In a recent study of a cluster of HSP, it was shown that the disease tended to occur in lower socioeconomic groups associated with prior sore throat.

HSP  (n=35) Skin lesions 35 Abdominal pain 20 Intussusception 1 Painful periarticular swelling 15 Hematuria 20 Abnormal RFT 2 Hypertension 3 Relapse 2 Chronic 3 Recurrences (>1 year) 2

leukocytoclastic angiitis typically - purpura slight focal necrosis and ulceration preferentially  lower extremities erythematous tender nodules livedo reticularis urticaria

Leucocytoclastic Vasculitis Fibrinoid necrosis of the vessel walls Infiltration of polymorphonuclear leukocytes Presence of nuclear debris Palpable purpura The pathologic infiltrate may change to predominantly mononuclear

Recluse Spiders Genus Loxosceles Family Sicariidae Six-eyed sicariid spiders Necrotic arachnidism Loxoscelism “gangrenous spot of Chile" At least 56 species One from the Mediterranean region Inside houses Hiding in the folds of clothing, shoes, or underneath boxes in storage rooms

Behcet’s syndrome Clinical features • Recurrent oral and/or genital aphthous ulceration • Chronic relapsing uveitis leading to blindness in 10% of all cases • A variety of skin manifestations, including the ‘pathergy’ phenomenon • Musculoskeletal, neurologic, major artery and vein involvement • An undulating course that generally abates in intensity with the passage of time

Behcet’s syndrome N=21 Mean age 12.1± 4.7 (6-16 y) Blindness 1 Severe eye damage 2 Meningoencephalitis 5 Pseudotumor cerebri 3 Aphthae 21 Genital ulcers 4 Arhtritis 10 DVT 1 Superficial vv thrombosis 3 Imuran 5 Cyclosporine A: Cyclophosphamide: chlorambucil 3

FMF Vasculitides in 3-11% Erysipelas like erythema = vasculitis? No increased prevalence of FMF in HSP pts