Vasculitis

Question 0

Medium vessels

Answer 0

Immune complexes : polyartheritis nodusa

Anti endothial cell antibody : Kawasaki

Question 1

Large vessel vasculitis

Answer 1

Granulomatous disease : giant cell , takayasu

Question 2

Small vessel

Answer 2

Microscopic polyangitis
Wegners granulomotosis
Chrug Strauss

Sle, IgA henoch schonlein, cryoglobulin, good pastures

Question 3

Pr3 - anca

Answer 3

C-anca

Wegners, microscopic poly, pan, Chrug, good pastures

Question 4

MPO- anca

Answer 4

P-anca

Primary vasculitis, microscopic polyangitis , Chrug, pan

Felty, sle, sjorgen, uc, crohns, primary sclerosing cholangitis

Question 5

Microscopic polyarteritis

Answer 5

Necrotising vasculitis affecting arteries, capillaries, venules

Glomerulonephritis and pulmonary capillaritis common ( not in PAN)

Question 6

Presents as palpable purpura

Answer 6

Microscopic polyarteritis

Question 7

Skin biopsy : lesions are all the same stage

Segmental fibrinoid necrosis of media

Answer 7

Microscopic polyarteritis

Question 8

Have fragmented pmns - leucocytoclasia

Answer 8

Microscopic polyarteritis

Question 9

Cause of microscopic polyarteritis

Answer 9

Reaction to antigens once antigen is removed it goes away

Question 10

Triad :acute necrotizing Granulomatous inflammation of medium to small vessels in upper and lower respiratory tract

Affects other body sites- eye and skin

Focal necrotizing glomerulonephritis

Answer 10

Wegners granuolmatosis

Question 11

Age of onset and test for Wegners

Answer 11

5th decade m>f and c-anca detectable in 95%

Question 12

Begins as severe type of asthma

Sinusitis ~>asthma ~> organ involvement

Answer 12

Churg Strauss

Question 13

Diagnostic markers for churg Strauss

Answer 13

Blood eosinophilia, eosinophils and granulomas in affected tissue and p- anca . Mainly affects lungs

Question 14

Segmental transmutation necrotizing vasculitis in medium and small arteries . Doesn’t affect arterioles, capillaries, venules

Aneruysms and thrombosis common

Answer 14

Polyarteritis nodosa

Question 15

Spares the lungs and if a disease of young adults ….m>f

Answer 15

PAN

Question 16

Clinical signs of PAN

Answer 16

Ischemia and infarction often episodic. And general symptoms of malaise, fever weightless, etc

30% have HBsAg in serum
Both c-ANCA and p-ANCA but p is more common

Question 17

Treatment pan

Answer 17

Untreated - fatal unless cutaneous

Corticosteroid and cyclophosphamide

Question 18

Vasculitis of children and infants parents as acute fever rash,viral erythema, red lips and strawberry tongue

Answer 18

Kawasaki

Question 19

Kawasaki diseases symptoms and problems

Answer 19

Asymptomatic to mi coronary artery aneurysms with rupture and death

Leading cause of acquired heart disease in the us and japan. Similar lesions as pan .

Cause is thought to be viral induced in genetically susceptible patients

Tx with anti- pyretics and IVIG

Question 20

Affects medium to to small vessels - mainly radial and tibial arteries ususally young male and women smokers

Answer 20

Buergers disease

Question 21

Buergers disease age and hla type

Answer 21

Before the age of 35
Hla -a9 and b5

Common in Japan, Israel, India

Hypersensitive to injected tobacco extract

Question 22

Inflammation of. Large medium and small arteries.

Usually affects head arteries ( temporal, ophthalmic, vertebral) or aorta

Answer 22

Giant cell (temporal) arteritis

Question 23

Most common vasculitis in adults. You see segmental modular thickenings, lumen is reduced.

Answer 23

Giant cell arteritis

Question 24

Giant cell arteritis clinical symptoms

Answer 24

``` Usually elderly 70-80 - half pts have visual symptoms - vague constitutional symptoms( fever, fatigue, weightloss, HA) Facial pain over the temporal artery May cause thoracic aortic aneurysm ```

Question 25

What do you see on biopsy giant cell

Answer 25

Fragmentation of internal elastic membrane Granulomatous inflammation of emerita near the internal elastic membrane Multinucleated giant cells

Question 26

Granulomatous inflammation of large to medium sized arteries. Fibrous thickening of aortic arch and it's branches and in half of cases the pulmonary arteries are affected

Answer 26

Takayasu arteritis

Question 27

Takayasu clincial symptoms and biopsy results

Answer 27

Females > 40 Wake pulses and low BP in upper extremities. Ocular probs , neuro deficits, pulm HTN, HTN, claudication. Narrowing and obliteration of lumens On angiogram Biopsy: intimate thickening, mononuclear inflammation, giant cells, lumens occluded by partially organized thrombus

Question 28

Vasculitis that look like micros poco polyarteritis and pan

Answer 28

RA, sle , henoch- schonlein purpura , mixed cryoglobulinemia, malignancy

Question 29

Henoch- schonlein purpura

Answer 29

Generalized vasculitis of small vessels of the skin (purpura), GI tract, joints (arthritis), kidneys (rarely - lungs & CNS) Most common vasculitis in children. 75% of cases occur in children 2-11 years old. Rare in adults. IgA antibody– antigen complexes ĺ deposit locally - throughout bodyand activate pathways leading to necrotizing vasculitis. Bacterial, viral, vaccine, drug, idiopathic causes. No single etiology has been clearly identified.

Question 30

Why is it important to differentiate sle from what

Answer 30

APLA syndrome because treatment is different steroids vs anti coagulation

Question 31

Anti phospholipid antibody (APLA) Hughes syndrome

Answer 31

3 different antibodies cause both arterial & venous thrombosis (blood clots). 1. anticardiolipin antibodies 2. antibodies directed against specific molecules (e.g. beta-2-glycoprotein 1). 3. lupus anticoagulant (LA) > 50% patients have primary APLAS. Prevalence in general population ~2-4%. Any age (esp. females with SLE) Secondary APLAS - ~ 30% of patients with SLE develop. Anticardiolipin antibodies are more common than lupus anticoagulant (~ 5X) In patients with 1º APLAS, ~ 10% will eventually be diagnosed with an autoimmune disorder (e.g. SLE or a mixed connective tissue disorder). Antibodies induce a thrombophilic state. Exact mechanism is unknown. APLA react will endothelial cell membrane phospholipids ĺ cell damage ĺ thrombosis APLAS is associated with miscarriages, preterm labor, preeclampsia, and eclampsia. Thrombocytopenia (low platelets) observed in 20-40% of patients. Stroke, MI, kidney damage, PE If multiple clots – catastrophic APS (CAPS)

Question 32

Red papule forms a vesicle in the center. Dries, forms a black scab. No scarring. ``` Papule is usually on face, trunk, extremities. Palms, soles, and oral mucosa may be involved. ``` Papule precedes fever.Serology.

Answer 32

Rickettsialpox - R. akari

Question 33

Papule becomes a vesicle on an erythematous base. Resembles a "dew drop” on a rose petal. Papule begins on head ĺ progresses to trunk, arms, then legs. Vesicles present in all stages. Common in children. Positive Tzanck test or DFA.

Answer 33

Chicken pox (VZV)

Question 34

``` Red macule or papule with central vesiculation surrounded by a red areola. Papule crusts in a few days. No scarring. ``` Palmar surfaces and sides of fingers, hands, and plantar toe skin. After fever, in 1-2 days oral ulcers on the hard palate, tongue, buccal mucosa. Cell culture & PCR. RX: symptomatic relief

Answer 34

Hand-foot-and-mouth Disease (enteroviruses, e.g. coxsackievirus A16)

Question 35

``` At tick bite site, an ulcer with a red halo forms. Later becomes generalized maculopapular rash. Rash is generalized, involves palms and soles, and is rarely hemorrhagic. ``` Fever, headache, and generalized lymphadenopathy Symptoms similar to a milder form of RMSF. Serology – R. conorii Ab

Answer 35

Boutonneuse fever

Question 36

At mite bite site, vesicle or black scab appears on an erythematous base. Vesicles are usually on trunk or extremities. Rash fades within a few days.Pneumonitis is frequent. Serology.

Answer 36

Scrub typhus - o. Tsutsugamishi

Question 37

Louse-borne typhus | epidemic

Answer 37

R. prowazekii | Human body louse (Pediculus humanus)

Question 38

Murine typus

Answer 38

R. typhi Rat flea (also endemic CA, HI, TX)

Question 39

African tick bite fever

Answer 39

R. africae | Buffalo ticks - usually live on cattle

Question 40

Scrub typhus

Answer 40

O. tsutsugamushi | Chigger mite

Question 41

Hands and feet then to trunk

Answer 41

Rmsf

Question 42

Extremities to trunk

Answer 42

Small pox and kawasakis

Question 43

Lower extremities

Answer 43

N. Meningitidis

Question 44

Hair line and downward

Answer 44

Measles

Question 45

Head to trunk to extremities

Answer 45

Varicella

Question 46

Neck to trunk

Answer 46

Scarlet fever group a strep s. Pyogenes

Question 47

Localized or generalized skin desquamation

Answer 47

Staph aureus, scalded skin syndrome. Exfoliatins toxin

Question 48

Scarlatina like rash that desquamates

Answer 48

Staph aureus TSS . Toxic shock toxin (tsst 1 )

Question 49

Cowdry type a inclusions

Answer 49

Intranuclear - VZV - herpes zoster

Question 50

Molluscum contagiosum

Answer 50

Benign papular eruption of multiple umbilical end cutaneous lesions

Question 51

H and e stain of Molluscum contagiosum

Answer 51

Epidermal acanthosis (hyperplasia) - MC grows into dermis to form multiple lobules. Eosinophilic intra-cytoplasmic inclusion bodies - at the lobule base, more basophilic at superficial layer. Central crater - into which the inclusion bodies discharge their contents.

Question 52

Intracellular growth

Answer 52

Rickettsia (RMSF, typhus), VZV, HSV, MCV, rubella

Question 53

Endotoxin

Answer 53

Neisseria meningitidis | LPS causes vasculitis & DIC

Question 54

Exotoxin

Answer 54

Streptococcus pyogenes (Group A Strep.)Pyrogenic (erythrogenic) toxins A-C

Question 55

Bacteria in vessel wall

Answer 55

Treponema pallidum endothelial proliferation lymphoplasmocytic vasculitis endarteritis obliterans (fibrosis/healing)

Question 56

Immune Attack | of infected cells

Answer 56

Measles (Rubeola) T cells attack viral antigen in infected endothelium of small vessels ĺ Koplick Spots:(oral lesions on mucous membrane enanthem)

Question 57

Complement Activation | [Immune / Autoimmune]

Answer 57

Cutaneous Necrotizing Vasculitis: Microscopic polyarteritis (small vessels) Giant Cell Arteritis (large temporal art. & aorta) Polyarteritis Nodosa (PAN) (medium & small) 91

Question 58

Petechia rash | Fulminant disease is Waterhouse friderichsen syndrome what is that

Answer 58

N. Meningitidis ``` non-palpable purpura ecchymosis shock DIC adrenal gland destruction Grey/black necrosis with DIC. ```

Question 59

Septic arthritis ĺ most common initial manifestation (migratory polyarthralgia). Rash ĺ occurs below the neck (spares scalp, face, mouth). Small papules or petechiae located on trunk or extremities, palms and soles.- turn into pustules on broad erythematous bases and necrotic centers. 9

Answer 59

Neisseria Gonorrhoeae

Question 60

Impetigo

Answer 60

Vesicles that crusts Strep pyogenes, staph aureus

Question 61

Erysipelas

Answer 61

Superficial cellulitis variant Margin sharply demarcated, painful Step. Pyogenes ans staph aureus Spreads via lymphatics

Question 62

Gumma

Answer 62

Syphilis

Question 63

1° Tiny red papule on legs and other exposed areas, becomes an elevated erythematous plaque. Satellite papules form after few months; may fuse together. No ulceration (unlike syphilis). 2° 5 -12 months after 1° stage, erythematous circular patcheson extremities and face; may go unnoticed. 3° Lesions with blue pigmentation on face, head, neck. Replaced by depigmented spots resembling vitiligo.

Answer 63

T. Careteum -'blue stain disease

Question 64

1°Skin papules develop on extremities ~ 1 month after infection, Enlarge to form papillomatous “frambesiform” nodules. Usually children are affected. 2 Yellow crusted granulomatous nodules appear on extremities and face and usually heal; may reoccur. 3 Long chronic course of remissions and relapses. Systemic symptoms ~>fever, malaise, LN May have skin destruction ~>large ulcerated nodules Disfiguration esp. around nose ~>gangosa Painful open sores on feet soles~> crab yaws Later, bone destruction ~>tibias

Answer 64

Tp subsp. Pertenue

Question 65

When does the rash appear with measles

Answer 65

4th febrile day . Hair line to toes in 2-3 days . Maculopapular