Title: GZ / The Grenz Zone Season 2 / 29 Vasculitis II
Tags: GZ DermnetNZ Waikato

Q: C-ANCA: cytoplasmic ANCA antibodies that target [...]
P-ANCA: perinuclear ANCA antibodies that target [...]
A: proteinase 3; myeloperoxidase

Q: What is the classic triad for Granulomatosis w/ polyangiitis (GPA) aka Wegener's
[...], [...], and [...]
A: Upper respiratory; lower respiratory; renal changes
Back Extra: Sinusitis, strawberry gums, or oral ulcers
Lower respiratory signs may include cough, SOB, or hemoptysis
Renal changes often present as a severe glomerulonephritis with hematuria

Q: What skin changes are associated with granulomatosis with polyangiitis?
[...], [...]-like ulcers, or [...]
A: Palpable purpura; pyoderma-gangrenosum; sub-Q nodules
Back Extra: May also present with peripheral neuropathy, stroke-like sx, systemic fevers, anorexia, & arthralgias in addition to classic triad of renal, upper and lower respiratory system changes

Q: Patients with granulomatosis with polyangiitis often present with a positive [...]-ANCA and an elevated ESR
A: c
Back Extra: Aka cytoplasmic ANCA that targets proteinase 3

Q: What is the most deadly manifestion of granulomatosis with polyangiitis?
[...]
A: Glomerulonephritis
Back Extra: Mortality rate of 80% if patients aren't treated in the first year (with cyclophosphamide, prednisone, or other immunosuppressants)

Q: Biopsy of granulomatosis with polyangiitis would show [...] along with necrotizing [...]
A: leukocytoclastic vasculitis; palisading granulomas

Q: Simply put, microscopic polyangiitis is similar to GPA but presents without the [...] changes or [...]
A: upper respiratory; granulomas
Back Extra: Changes to lower respiratory system and renal system occur, as in GPA

Q: Buzz words for a certain vasculitis
Positive [...]-anca, lower respiratory changes, glomerulonephritis with sparing of the upper respiratory system?
[...]
A: p; Microscopic polyangiitis
Back Extra: Simply put, MPA is like GPA (with lower respiratory and renal changes) but without the upper resp changes + granulomas

Q: Microscopic polyangiitis may present with [...], which refers to inflammation of two or more nerve areas resulting in sensory or motor symptoms
A: mononeuritis multiplex
Back Extra: Also seen in Churg-Strauss and other vasculitides

Q: Buzz words for a certain vasculitis
Adult onset asthma, eosinophilia, [...]-anca
[...]
A: p; Eosinophilic granulomatosis w/polyangiitis
Back Extra: Aka Churg-Strauss
PEARL: EGPA does NOT have renal changes, which differentiates it from GPA & MPA
Presents in 3 stages
1. Adult-onset asthma, allergic rhinitis, nasal polyps
2. Eosinophilia, pneumonia, GI issues
3. Systemic vasculitis w/ palpable purpura, worsening asthma & allergic rhinitis, and mononeuritis multiplex & cardiac issues (e.g. cardiomyopathy)

Q: Leading cause of mortality for patients with eosinophilic granulomatosis with polyangiitis?
[...]
A: Cardiomyopathy
Back Extra: Presents in 3 stages
1. Adult-onset asthma, allergic rhinitis, nasal polyps
2. Eosinophilia, pneumonia, GI issues
3. Systemic vasculitis w/ palpable purpura, worsening asthma & allergic rhinitis, and mononeuritis multiplex & cardiac issues (e.g. cardiomyopathy)

Q: [...], [...], and [...] may also produce positive ANCA antibodies, so don't automatically assume that p-anca or c-anca infers that a patient has one of the ANCA-associated vasculitides
A: Minocycline; levamisole tainted cocaine; hydralazine
Back Extra: In addition, there may be crossover in the 3 ANCA-associated vasculitides (MGPA with c-ANCA rather than the classic p-ANCA

Q: Buzz words for a certain vasculitis
Hepatitis B, medium vessel involvement, testicular pain
[...]
A: Polyarteritis nodosa
Back Extra: May be cutaneous or systemic, patients may present with orchitis
Criteria (> 3 or more of these 10)
1. Weight loss of 4kg or more
2. Livedo reticularis
3. Testicular pain/tenderness
4. Myalgias or leg weakness or tenderness
5. One or more neuropathies.
6. Hypertension with diastolic blood pressure >90
7. Elevated BUN >40 or creatinine >1.5 that can’t be
explained by dehydration or obstruction.
8. Positive hepatitis B antigen or antibodies.
9. Arteriogram demonstrating aneurysms or occlusion of
visceral arteries without another explanation.
10. Biopsy showing small or medium-sized vessel with
inflammation (including PMN’s)

Q: What organ system is usually spared in polyarteritis nodosa?
[...]
A: Lungs

Q: Cutaneous findings for polyarteritis nodosa may include subcutaneous nodules that travel along the course of blood vessels along with [...]
A: livedo reticularis

Q: Why are patients with polyarteritis nodosa not affected by glomerulonephritis?
[...]
A: PAN affects medium-sized vessels that perfuse the kidneys, NOT the small vessels w/in the glomeruli
Back Extra: Present with hypertension but NOT glomerulonephritis

The associations to remember with polyarteritis nodosa include Hepatitis B > C, HIV, CMV, strep infections, and inflammatory bowel disease

Biopsy of polyarteritis nodosa may show leukocytoclastic vasculitis w/in medium-sized arteries in the deep dermis or sub-Q, along with a lobular panniculitis next to the involved vessels

Q: Patients with polyarteritis nodosa should undergo a(n) [...] to look for aneurysms or renal artery stenosis
A: renal angiogram

Q: Buzz words for a certain vasculitis:
Child, fever, strawberry tongue
[...]
A: Kawasaki disease

Q: How do you treat a patient with Kawasaki disease?
For acutely ill, give [...] and [...]
A: IVIG; Aspirin
Back Extra: IVIG @ 2g/kg over 12 hrs as a single dose
Aspirin @ 80-100 mg/kg daily

Q: Whenever you administer IVIG to a patient, why would you check IgA levels?
[...]
A: IVIG contains all antibodies, and patients who are IgA deficient may develop an anaphylactic response against those antibodies
Back Extra: Selective IgA deficiency is relatively common

Q: Which vasculitis may present with headache, jaw claudication, polymyalgia rheumatica, and visual changes?
[...]
A: Temporal Arteritis (Giant Cell Arteritis)
Back Extra: Workup involves ESR, CRP, temporal artery biopsy
Skin Changes may include erythema, cyanosis, purpura, or tender nodules

Q: Which vasculitis may present in younger patients with constitutional symptoms and radial pulses that carry a difference in blood pressure >10 mmHg between each arm?
[...]
A: Takayasu arteritis
Back Extra: Constitutional sx: fevers, night sweats, and weight loss, claudication of the extremities
Skin changes: erythematous papules, purpura, erythema nodosum, and Raynaud’s phenomenon
Tx with prednisone

Septic vasculitis may have several causes, including subacute bacterial endocarditis, Staphylococcal or Pseudomonas septicemia, Neisserial gonococcemia and meningococcemia, Vibrio vulnificus, rickettsialpox and Rocky Mountain Spotted fever
Back Extra: Presentation of septic vasculitis can vary widely, from hemorrhagic pustules of staph septicemia to large ulcerating bullae caused by pseudomonas in cases of ecthyma gangrenosum
Septic vasculitis is caused by systemic infection that damages the vessels by either 1) invading the vessels directly or 2) causing immune- mediated damage

Q: Histology of septic vasculitis?
[...] vessel vasculitis with [...] and [...]
A: Small; neutrophils; thrombi
Back Extra: PEARL: Septic Vasculitis histology differs from the vasculopathies in that vasculopathies like DIC and TTP have minimal or no inflammatory infiltrate to go with their thrombi on path, while septic vasculitis does have inflammation with thrombi!