17.Αγγείτιιδες (εκτός πορφύρα, δικτυωτή πελίδνωση) [550-569]

Question 1

Name three clinical findings commonly associated with temporal (giant cell) arteritis

Answer 1

Unilateral headache (temporal artery), jaw claudication, and impaired vision/irreversible blindness

Question 2

Which MPO-ANCA/p-ANCA7#8211;positive vasculitis is a granulomatous vasculitis with eosinophilia?

Answer 2

Churg-Strauss syndrome (also called eosinophilic granulomatosis with polyangiitis)

Question 3

A 60-y/o woman with polymyalgia rheumatica has a unilateral headache and jaw claudication. What disease process may cause blindness in her?

Answer 3

She is most likely experiencing temporal arteritis, in which occlusion of the ophthalmic artery can result in irreversible blindness

Question 4

Patients with temporal arteritis may also have which rheumatologic condition? What population is the most commonly affected?

Answer 4

Polymyalgia rheumatica; elderly women

Question 5

In Churg-Strauss syndrome, the patient will test positive for ____ (p-ANCA/c-ANCA) in serum.

Answer 5

p-ANCA (MPO-ANCA)—usually has an elevated IgE level

Question 6

Temporal arteritis is associated with what laboratory test results? What is the treatment?

Answer 6

Elevated erythrocyte sedimentation rate; high-dose steroids prior to biopsy prevent blindness

Question 7

All ANCA-positive vasculitides affect ____ (small/medium/large) vessels.

Answer 7

Small

Question 8

A 78-y/o woman with polymyalgia rheumatica presents with an intense temporal headache. What types of arteries are likely affected?

Answer 8

Large arteries, usually branches of the carotid artery (she has temporal arteritis)

Question 9

What is the most common childhood systemic vasculitis? It often presents after what disease?

Answer 9

Henoch-Schönlein purpura; typically follows upper respiratory infections

Question 10

A 7-year-old girl has arthralgias, abdominal pain, and purpura on her legs after an upper respiratory infection. Pathophysiology?

Answer 10

Associated with IgA nephropathy (the patient has Henoch-Schönlein purpura, a small-vessel vasculitis 2° to IgA immune complex deposition)

Question 11

What is the triad of Henoch-Schönlein purpura?

Answer 11

Palpable purpura on the buttocks and legs, arthralgias, and abdominal pain

Question 12

Takayasu arteritis is associated with what laboratory finding? What histologic findings are seen on biopsy of affected vessels?

Answer 12

An elevated erythrocyte sedimentation rate; one sees granulomatous thickening and narrowing of affected vessels

Question 13

What are the symptoms of Takayasu arteritis? What is the most affected demographic?

Answer 13

Fever, night sweats, arthritis, weak upper extremity pulses, myalgias, skin nodules, ocular disturbances; Asian females younger than 40

Question 14

What is polyarteritis nodosa?

Answer 14

Immune complex–induced necrosis of medium-sized arteries typically involving the renal and visceral vessels (but not pulmonary arteries)

Question 15

Name 10 clinical findings associated with polyarteritis nodosa.

Answer 15

Fever, weight loss, malaise, abdominal pain, melena, headache, hypertension, neurologic dysfunction, cutaneous eruption, renal damage

Question 16

Polyarteritis nodosa is strongly associated with which infectious disease?

Answer 16

Hepatitis B (30% of patients are seropositive)

Question 17

Polyarteritis nodosa most commonly affects which population? Describe its histologic appearance.

Answer 17

Young adults; transmural inflammation of arterial wall with fibrinoid necrosis due to immune complex deposition

Question 18

Fever and which five clinical findings are associated with Kawasaki disease?

Answer 18

Conjunctival injection, Rash, cervical Adenopathy, Strawberry tongue (mucositis), Hand/foot changes (edema/erythema), & fever(CRASH & burn)

Question 19

What is Buerger disease?

Answer 19

A thrombosing vasculitis affecting the medium-sized peripheral arteries and veins of heavy smokers (usually men &60;40 years of age)

Question 20

Buerger disease goes by what other name?

Answer 20

Thromboangiitis obliterans

Question 21

What are three clinical findings associated with Buerger disease? What would a biopsy of an affected artery show?

Answer 21

Intermittent claudication, superficial nodular phlebitis, and Raynaud phenomenon; histology shows segmental thrombosing vasculitis

Question 22

What are the two main presentations of microscopic polyangiitis? Treatment?

Answer 22

Pauci-immune glomerulonephritis and palpable purpura (involves lungs, kidneys, and skin); treat with cyclophosphamide and corticosteroids

Question 23

What is granulomatosis with polyangiitis (Wegener granulomatosis)?

Answer 23

A small-vessel vasculitis characterized by focal necrotizing vasculitis, granulomas in the lung and upper airway, and glomerulonephritis

Question 24

A patient with Wegener granulomatosis may have which upper respiratory tract symptoms? Lower respiratory tract? Renal?

Answer 24

Perforation of nasal septum, chronic sinusitis, otitis media, mastoiditis; cough, dyspnea, hemoptysis; hematuria with red blood cell casts

Question 25

In Wegener granulomatosis, x-ray of the chest may reveal what finding?

Answer 25

Large nodular densities

Question 26

Wegener granulomatosis and microscopic polyangiitis have similar presentation. How can you differentiate between the two?

Answer 26

Wegener granulomatosis has nasopharyngeal involvement and necrotizing granulomas, whereas microscopic polyangiitis has neither

Question 27

Η κνιδωτική αγγειτιδα είν

Answer 27

κλινοπαθολογική οντότητα

Question 28

Κνιδωτική αγγείτιδα - κλινική εικόνα

Answer 28

Οι κνιδωτικές βλάβες διάρκούν περισσότερο από 24 ώρες και παρουσιάζουν πορφύρα και μεταφλεγμονώδη υπέχρωση και αίσθημα καύσου παρά κνησμού

Question 29

Κνιδωτική αγγείτιδα - αιτιο

Answer 29

Ιδιοπαθής
όταν υπάρχει χαμηλό συμπλήρωμα μπορει να συνυπάρχει άσθμα και συμμετοχή του γες
Sjogran k SLE
Ορονοσία

Question 30

Κνιδωτική αγγείτιδα - εργαστηριακά

Answer 30

Αυξ. Τ.Κ.Ε

Υποσυμπληρωματία, θετικά αντιπυρηνικά αντισώματα σε χαμηλό τίτλο και αιματουρία

Question 31

Κνιδωτική αγγείτιδα - Tx

Answer 31

Αντιισταμινκά για ήπιες περιπτώσεις
ΜΣΑΦ
Πρεδνιζολόνη
Δαψόνη
Κολχικίνη
\+υδροξυχλωροκίνη