Rheum 5 - Vasculitis

Question 1

Approaches to classification of vasculitis

Answer 1

Clinical syndromes
Vessel size - large arteries, medium and small mm arteries, small vessels (arterioles and venules)
Lab markers
Pathology - immune complex deposition, granulomatous inflammation

Question 2

Clinical features with vasculitis

Answer 2

Multisystem disease
Unexplained constitutional s/s
Skin lesions (palpable purpura)
Ischemic vascular changes 
Glomerulonephritis
Mononeuritis multiplex
Myalgia, arthralgia/arthritis
Abdominal or testicular pain

Question 3

Polyarteritis Nodosa (PAN)

Answer 3

Necrotizing vasculitis of small and medium arteries
Lesions are segmental
Favor vessel branch points
Different stages of development - simultaneously
Aneurysms

Question 4

Epidemiology - PAN

Answer 4

Uncommon!
Avg age onset = 40 to 60
M:F is 2:1

Higher incidence in pop with HBV

Question 5

PAN classification

Answer 5

Polyarteritis Nodosa

• Idiopathic
• Associated with cirus (primarily hep B)
• Associated with malignancy (mainly hematologic)

Question 6

ACR criteria for classification of PAN

Answer 6

Need at least 3 of the 10
1 Weight loss more than 4 Kg
2 Livedo reticularis 
3 Testicular pain or tenderness
4 Myalgias, weak, leg tenderness
5 Mono or polyneuropathy
6 Diastolic BP more than 90
7 Elevated BUN/creatinine
8 Hep B
9 Arteriographic abnormaiity
10 Biopsy of small or medium artery containing PMN

Question 7

PAN cutaneous

Answer 7

25-60% of patiens
Palpable purpura
Infarctions
Ulcerations
Livedo reticularis 
Subcutaneous nodules
Ischemic distal digits

Question 8

PAN neurologic

Answer 8

Up to 70%
LE more than UE
Mononeuritis multiplex (esp affecting superficial peroneal, sural, radial, cubital, median)
Symmetric polyneuropathy (sensory and motor) - stocking glove dysesthesia
CNS - HA, seizure, CN palsy, CVA

Question 9

PAN - GI

Answer 9

Abdominal pain = 25-70%
Intestinal angina
Bowel infarction
Gall bladder or appendix

One of the most sever manifestations - primary causes of death within first year (after infection and cardiac)

Question 10

PAN - renal

Answer 10

If classic PAN = glomerulus is usually spared 
Vascular neuropathy (35%
HTN - 25-30%
If glomerulonephritis = microscopic polyangitis (MPA)

Question 11

Labs for PAN

Answer 11

Elevated ESR 95%
Elevated WBC 75%
Anemia of chronic disease 67%
Depressed C3 70%
Pos RF 40% (not pos ANA)
Elevated C reactive protein
HebBSAg

Question 12

PAN diagnosis

Answer 12

Tissue diagnosis - sural nerve, testes, skeletal muscle, renal
Visceral angiography

Question 13

PAN tx

Answer 13

Corticosterois - prednisone
Cyclophosphamide (second choice azathioprine)
Control hyeprtension

Question 14

IgA Vasculitis (Henoch Scholein Purpura - clinical manifestations

Answer 14

Non thrombocytopenic purpura
Arthralgias/arthritis
GI pain - colicky abdominal pain; GI hemorrhage risk, Renal abnormalities

80-90% will have all four of these

Question 15

IgA Vasculitis (Henoch Scholein Purpura - classic tetrad in 80% of cases

Answer 15

1 palpable purpura
2 arthritis
3 abdominal pain
4 renal disease

Question 16

IgA Vasculitis (Henoch Scholein Purpura - clinical features - demographics

Answer 16

Incidence school age = 13/100,000
Develop at any age - 90% 4-7 yo
2/3 follow resp infection 
Immune complex deposition - IgA 1
F:M is 1:1
Uniphasic 80%, polyphasic 10-20%

Majority are treated quickly and resolve - those that linger tend to have renal involvement
Adults more likley to have severe disease with renal involvement

Question 17

IgA Vasculitis (Henoch Scholein Purpura - - Skin

Answer 17

Palpable purpura (100% prereq for diagnosis)
Usually LE - buttock
Presenting sign - 50%

Question 18

IgA Vasculitis (Henoch Scholein Purpura - - joint involvement

Answer 18

Arthralgias/arthritis (60-85%)
Knees more than ankles more than wrists more than elbows
Transient - self limiting; without permanent joint damage

Question 19

IgA Vasculitis (Henoch Scholein Purpura - - GI involvement

Answer 19

Colicky abdominal pan, nausea, vomiting - peds 70%, adults 50%
GI bleeding occult (50%)
Major hemorrhage (less than 5%)
Intussusception - ileoileal (mean age 6 yo)
Abdominal pain secondary to submucosal and intramural extravasation of fluid into intestinal wall

Question 20

IgA Vasculitis (Henoch Scholein Purpura - - renal involvement

Answer 20

Incidence 10-50%
Children usually more than 9 yo
Inc incidence of GMN with bloody stools
Less than 1% progress to ESRD

Question 21

IgA Vasculitis (Henoch Scholein Purpura - Lab

Answer 21

Elevated ESR 1/3
Platelet count normal
U/A microscopic hematuria, proteinuria, RBC cast
Elevated IgA 50%
Skin biopsy positive IF for IgA in vessel walls

Question 22

IgA Vasculitis (Henoch Scholein Purpura - - treatment

Answer 22

Supportive APAP (tyelenol) for analgesia 
Corticosteroids - abomdinal pain, nephritis, edema, ? benefit
Severe HSP nephritis - high dose corticosteroids, cyclophosphamide

Question 23

ANCA - associated vasculitis

Answer 23

1 Granulomatosis with polyantitis (Wegeners granulomatosis)
2 Eosinophilic granulomatosis with polyangitis (churg strauss syndrome)
3 Microscopic polyangitis

Question 24

ANCA is what

Answer 24

Antineutrophil cytoplasmic antibodies

Question 25

ANCA - Granulomatosis with polyangitis - Wegener's - GPA is what

Answer 25

``` Multi organ systemic disease Granulomas necrotizing vasculitis - predilection upper and lwoer resp tract and kidney Prevelance 3/100,000 M:F is 1:1 Age - 40 to 60 Mainly cauc Association with PR3 90-97% specific "saddle nose deformity" ```

Question 26

ANCA - Granulomatosis with polyangitis - Wegener's - GPA - Lungs

Answer 26

Trachea - subglottic stenosis Pulmonary - fleeting pulmonary infiltrates, ndoules, fulminant alveolar hemorrhage Asymptomatic, cough, dyspnea, pleurisy, hemoptysis

Question 27

ANCA - Granulomatosis with polyangitis - Wegener's - GPA - renal

Answer 27

``` RPGN Hematuria RBC cast Proteinuria Rising serum creatinine Can be rapidly progressive ```

Question 28

ANCA - Granulomatosis with polyangitis - Wegener's - GPA - Lab

Answer 28

``` Elevated ESR (often over 100) Leukocytosis Thrombocytosis Anemia Mildly positive RF - 60% Abnormal urinary sediment cANCA PR3 ```

Question 29

ANCA - Granulomatosis with polyangitis - Wegener's - GPA - Treatment

Answer 29

Prednisone Cyclophosphamide Rituxan Limited disease - prednisone and methotrexate

Question 30

ANCA - Granulomatosis with polyangitis - Wegener's - GPA - prognosis

Answer 30

Prednisone and CTS - 95% remission Remission relapse is 50% Mortality 8 yrs - 13%

Question 31

ANCA - microscopic polyangitis (MPA) - Typical manifestations

Answer 31

peak age 30-50 Frequent manifestations: Pulmonary capillaries/diffuse alveolar hemorrhage Necrotizing glomerulonephritis (almost always) So mainly lung and kidney involvement

Question 32

ANCA - microscopic polyangitis (MPA) - Other manifestations

Answer 32

Fever (50-70%) Arthralgias (30-65%) Pupura (40%) Peripheral or CNS (25-30%)

Question 33

ANCA - microscopic polyangitis (MPA) - Histopathology of MPA

Answer 33

Focal, segmental necrotizing GN Pauci immune (no immune deposits) Lung capillaritis Skin leukocytoclastic vasculitis

Question 34

ANCA - microscopic polyangitis (MPA) - diagnosis

Answer 34

Clinical features Biopsy results Lab data - P ANCA against MPO in 80% (PANCA is bound around MPO)

Question 35

ANCA - Eosinophilic granulomatosis with polyangiitis (churg strauss syndrome)

Answer 35

Granulomatous inflammation Small and medium vessels Peripheral eosinophilia Primarily with a prior history of allergic manifestations - Rhinitis (often with nasal plyps), Adult onset asthma

Question 36

ANCA - Eosinophilic granulomatosis with polyangiitis (churg strauss syndrome) - clinical pahses

Answer 36

1 Prodromal phase - allergic manifestations - fora decade or more 2 Peripheral blood and tissue eosinophilia 3 Life threatening systemic vasculitis

Question 37

ANCA - Eosinophilic granulomatosis with polyangiitis (churg strauss syndrome) - clinical features

Answer 37

Paranasal sinus - sinusitis, rhinitis, plyps Lungs - patchy, shifting infiltrates, nodules, effusion NS - mononeuritis multiplex, polyneuropathy Skin - nodules, purpura, infarction Joints GI - pain, bloody, diarrhea, masses CHF Misc - renal is RARE

Question 38

ANCA - Eosinophilic granulomatosis with polyangiitis (churg strauss syndrome) - diagnosis

Answer 38

Clinical and pathologic features ANCA in 60-70% - primarily vs. MPO with PANCA pattern Diagnosis made based on clinical hx and often bipsy confirmation

Question 39

Large vessel vasculitis

Answer 39

Giant cell arteritis | Takayasu Arteritis

Question 40

Large vessel vasculitis - Giant cell arteritis (GCA) - Temporal arteritis

Answer 40

Temporal region - forehead If can;t feel pulses is more concerning Might have tenderness to palaption too

Question 41

Large vessel vasculitis - Giant cell arteritis (GCA) - epidemiology

Answer 41

Avg age 70 3:1 F more than M Caucasion almost exclusively

Question 42

Large vessel vasculitis - Giant cell arteritis (GCA) - GCA PMR Syndrome

Answer 42

Cranial arteriitis - HA, scalp tenderness, optic neuropathy, jaw claudication, PMR Large vessel invovlement - limb claudication, unequal pulses, aortic aneur, aoritc insuff, PMR System inflamm disease - fever, chills, anorexia, weight loss, night sweats, weak, depressed Isolated PMR - pain in shld and pelvic girdle mm, stiffness

Question 43

Relationship of polymyalgia (PMR) to giant cell arteritis

Answer 43

40-60% of pts with GCA have PMR symptoms - in about 1/2 of those, PMR is their first manifestation of GCA 10-15% of pts with PMR have GCA PMR symptoms can occur before, with , or after GCA symptoms in those with GCA

Question 44

Polymyalgia rheumatica epidemiology

Answer 44

Onset after 60 Norther europeans 2:1 F more than M

Question 45

Polymyalgia rheumatica - clincial and lab findings

Answer 45

``` Pelvic and shoulder girdlle aching Morning stiffness Rapid response to low dose of corticoseteroids Anemia Elevated ESR and C reactive protein ```

Question 46

GCA symptoms

Answer 46

``` HA Fever PMR Weight loss Anorexia Jaw claudications Malaise Depression Fatigue Acute visual loss Diplopia ```

Question 47

GCA vessel distribution

Answer 47

Cranial - superficial tempral - vertebral - ophthalmic - post ciliary - less ffrequnty in external and internal carotid Less common but inc recognized in aorta and branches, and cornary (rare)

Question 48

GCA - lab

Answer 48

``` Elevated ESR - either more than 50 or 100 Elevated C reactive protein Anemia of chronic disease Abnormal liver function test Elevated IL 6 SPEP inc alpha 2 globulin ```

Question 49

GCA - temporal artery biopsy

Answer 49

Should do | Obtain ASAP - though do not delay starting steroids prior to biopsy if suspicion is high

Question 50

GCA - predictors for a positive temporal artery bipsy

Answer 50

Positive predictors = jaw claduication, diplopia, TA beading, TA prominence, TA tenderness Neg = Absence of TA abnormality, normal ESR

Question 51

GCA - histopathology

Answer 51

Usually extracranial arteries Panarteries - invovles intima, destroys internal elastic membrane Granulomatous arteritis; inc multinucleated giant cells Skip lesions

Question 52

ACR classification criteria for GCA

Answer 52

NEED 3/5 ``` 1 Age over 50 at disease onset 2 new HA 3 Termpoal artery abnormality 4 Elevated ESR over 50 5 Abnormal biopsy ```

Question 53

GCA tx

Answer 53

``` Mandatory - prednisone for 4 wks Slow taper, thereafter Lower maitenance doeses of prednisone Total tx duration is 6 to 24 months Relpase is common ```

Question 54

Follow up of GCA pts

Answer 54

Vascular complications may occur years after onset Stenosis or aneurysm Aortic aneur is 17x mroe likley in those with GCA also 2.4x mroe likely to develop abdominal aneurysm