Rheum 5 - Vasculitis Flashcards
Approaches to classification of vasculitis
Clinical syndromes
Vessel size - large arteries, medium and small mm arteries, small vessels (arterioles and venules)
Lab markers
Pathology - immune complex deposition, granulomatous inflammation
Clinical features with vasculitis
Multisystem disease Unexplained constitutional s/s Skin lesions (palpable purpura) Ischemic vascular changes Glomerulonephritis Mononeuritis multiplex Myalgia, arthralgia/arthritis Abdominal or testicular pain
Polyarteritis Nodosa (PAN)
Necrotizing vasculitis of small and medium arteries
Lesions are segmental
Favor vessel branch points
Different stages of development - simultaneously
Aneurysms
Epidemiology - PAN
Uncommon!
Avg age onset = 40 to 60
M:F is 2:1
Higher incidence in pop with HBV
PAN classification
Polyarteritis Nodosa
- Idiopathic
- Associated with cirus (primarily hep B)
- Associated with malignancy (mainly hematologic)
ACR criteria for classification of PAN
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
PAN cutaneous
25-60% of patiens Palpable purpura Infarctions Ulcerations Livedo reticularis Subcutaneous nodules Ischemic distal digits
PAN neurologic
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
PAN - GI
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)
PAN - renal
If classic PAN = glomerulus is usually spared Vascular neuropathy (35% HTN - 25-30% If glomerulonephritis = microscopic polyangitis (MPA)
Labs for PAN
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
PAN diagnosis
Tissue diagnosis - sural nerve, testes, skeletal muscle, renal
Visceral angiography
PAN tx
Corticosterois - prednisone
Cyclophosphamide (second choice azathioprine)
Control hyeprtension
IgA Vasculitis (Henoch Scholein Purpura - clinical manifestations
Non thrombocytopenic purpura
Arthralgias/arthritis
GI pain - colicky abdominal pain; GI hemorrhage risk, Renal abnormalities
80-90% will have all four of these
IgA Vasculitis (Henoch Scholein Purpura - classic tetrad in 80% of cases
1 palpable purpura
2 arthritis
3 abdominal pain
4 renal disease
IgA Vasculitis (Henoch Scholein Purpura - clinical features - demographics
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
IgA Vasculitis (Henoch Scholein Purpura - - Skin
Palpable purpura (100% prereq for diagnosis)
Usually LE - buttock
Presenting sign - 50%
IgA Vasculitis (Henoch Scholein Purpura - - joint involvement
Arthralgias/arthritis (60-85%)
Knees more than ankles more than wrists more than elbows
Transient - self limiting; without permanent joint damage
IgA Vasculitis (Henoch Scholein Purpura - - GI involvement
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
IgA Vasculitis (Henoch Scholein Purpura - - renal involvement
Incidence 10-50%
Children usually more than 9 yo
Inc incidence of GMN with bloody stools
Less than 1% progress to ESRD
IgA Vasculitis (Henoch Scholein Purpura - Lab
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