What is Henoch-Schonlein Purpura?
- Small vessel vasculitis due to IgA complex deposition with dependent rash, bloody diarrhea, glomnephritis, arthralgias
- Most common vasculitis in children and occurs after URI
What are Sx of Henoch-Schonlein Purpura?
- Palpable purpura on buttocks and legs, Stellate-shapted ulcers, nodules, ischemic digits, livido reticularis, or GI pain and bloody diarrhea, Hematuruia (due to IgA nephropathy). Usually occurs following an upper respiratory infection
- Dx diascopy (blanch test) for DDx extravasated blood from dilation and statis
What is Tx for Henoch-Schonlein Purpura?
• Disease is usually self-limited, but may require steroids
What are the biomarkers for:
• Wegener Granulomatosis
• Microscopic Polyagiitis
• Churg-Straus Syndrom
- Wegener Granulomatosis ==> C-ANCA
- Microscopic Polyagiitis ==> P-ANCA
- Churg-Straus Syndrom ==> P-ANCA
What are the Large Vessel Vasculitis?
- Temporal (Giant Cell) Arteritis
* Takayasu Arteritis
What are the Medium-Vessel Vasculitis diseases?
- Polyarteritis nodosa
- Kawasaki Disease
- Buerger Disease
What are the small vessel vasculitis diseases?
- Wegener Granulomatosis
- Microscopic polyangiitis
- Churg-Straus Syndrome
- Henoch-Scholein Purpura
What is Temporal (Giant cell) Arteritis?
What age group is affected?
Are males or females more commonly affected?
- Granulomatous vasculitis that classically involves branches of the carotid artery
- mononuclear cell infiltrate destroys the elastic lamina of muscular arteries, w/ become tender, non-elastic and non-pulsatile
- Most common vasculitis in > 50 years old
- More common in females
What are the symptoms of Giant cell arteritis?
What are the causes of these symptoms?
- headache ==> temporal artery involvement (artery is also tender)
- visual disturbances ==> ophthalmic artery
- jaw claudication
- Flu symptoms and muscle pain (polymyalgia rheumatica) may be present.
- ↑ Erythrocyte sedimentation rate
- may present as wasting febrile disease
What is the Tx for Temporal Cell Arteritis?
What are risks of not treating?
- Corticosteroids
* Blindness may occur without treatment.
What is Takayasu Arteritis? In whom is it common? What are symptoms? What is Tx? What are differences in symptoms in the initial phase vs later stages?
- Granulomatous vasculities that involves the aoritic arch at branch points
- Presents in < 50, especially young asian females
- Visual and neurological symptoms with weak or absent pulse in upper extremity
- ↑ Erythrocyte Sedimentation Rate
- Tx: Corticosteroids
- While the initial phase is quite inflammatory with fever, malaise, weight loss and ischemic symptoms, the later stages of the disease are associated with progressive vascular occlusion without signs of inflammation systemically.
Compare Temporal (Giant Cell) Arteritis and Takayasu Arteritis
- TGCA > 50 y/o, carotid arteries
* TA < 50 asian women, aortic branch points, with systemic Sx
What is Polyarteritis Nodosa?
Who is affected?
What are the symptoms?
What virus is it associated with?
• necrotizing vasculitis involving multiple organs, sparing the lungs
• Classically presents in young adults as
o hypertension (renal artery involvement)
o Abdominal pain with melena (mesentertic artery)
o Neurological disturbances
o Skin lesions
• Hep B ==> HBsAg (CODE WORD)
• (similar histology is seen in RA, lupus, and Kawasaki)
What are the string of pearls appearance of Polyarteritis Nodosa?
- lesions of varying stages
- Early lesion ==> transmural inflammation with fibrinoid necrosis (fig 7.3)
- Later lesion ==> fibrosis
- Fibrosis causes string of pearls appearance
What is Tx of Polyarteritis nodosa?
What are consequences of not treating?
- Corticosteroids and cyclophosphamide
* Fatal if not treated.
What is Kawasaki Disease? What demographic? What vessels are involved? What are the risks? What are symptoms? What is treatment?
• Asian < 4 years old • Coronary artery involvement is common o Risks of thrombosis with MI o Aneurysm with rupture • Non-specific symptoms including o Mucosal inflammation o Fever o Conjunctivitis o Erythematous rash on palms and soles o Enlarged cervical lymph • Tx is aspirin and IVIg • Disease is self-limited, but may have coronary arteritis
What is Buerger Disease?
What are the presentation and symptoms?
What behaviors are risk factors?
- necrotizing vasculitis involving digits
- ulceration, gangrene, and autoamputation of fingers and toes.
- Raynaud phenomenon is often present
- smoking is causative
What is the Classic Triad of Wegeners?
vasculitis and extravascular granulomatous inflammation in the
o Upper airway (nose, sinuses, ears)
o Lower airway (lungs, trachea)
o Kidney (pauci-immune GNitis).
What is Wegener Granulomatosis? What is the demographic? What are the symptoms? What is the serum biomarker? What does biopsy reveal? What is Tx?
- necrotizing granulomatous vasculitis involving the nasopharynx, lungs, and KDs
- Middle aged men with sinusitis or nasopharyngeal ulceration, hemoptysis with bilateral nodular lung infiltrates, and hematuria due to glomerulonephritis
- Serum c-ANCA levels are biomarker
- Biopsy: large necrotizing granulomas with adjacent necrotizing vasculitis (see figure 7.4)
- C-ANCA
- Tx is cyclophosphamide and steroids (relapses are common)
What is Microscopic Polyagiitis?
What organs are involved?
What is biomarker?
What is Tx?
- necrotizing vasculitis involving multiple organs, especially the lungs and KDs
- Similar to Wegener granulomatosis, but nasopharyngeal involvement and granulomas are ABSENT
- Serum p-ANCA are biometric
- Tx is corticosteroids and cyclophosphamide (relapses are common)
What is Chrug-Straus syndrome?
What immune cells are involved?
What illnesses are associated?
What biomarker is used?
- necrotizing granulomatous inflammation with EOSINOPHILS involving multiple organs especially the LUNGS and HEART
- Asthma and peripheral eosinophilia
- P-ANCA is biomarker
What are the differences between Polyarteritis Nodosa vs Wegener’s Granulomatous vs Microscopic Polyagiitis vs Churg-Straus Syndrome in terms of organs involved and size of vessels involved?
- All: multiple organ involvement
- Poly: Med vessels. LG are spared
- Wegeners: Small vessels. Nasopharynx, LG, and KD
- Micro: Small vessels. LG and KD are targeted
- Churg: Small vessels. LG and HT are targeted. Has eosinophils.
What is Raynaud’s
• Involves digital vessels in fingers and toes, sometimes tip of nose and ears
• 1° (dz) is more common in young ♀, 2° (phenomen-on) is more common in adult ♀/♂, other dz (systemic sclerosis, CREST, SLE, DM, Smoking!!)
exaggerated vasomotor response to cold or stress
What is the clinical presentation of Raynaud’s in primary and secondary dz?
1°: Paroxysmal digital color Δ (white-blue-red sequence). Ulceration and gangrene in chronic cases
2°: systemic sclerosis and CREST – digital vasculitis w/ vessel fibrosis, dystrophic calcific-ation, ulceration, gangrene
