230: Vasculitis Flashcards
What is the treatment for anti-GBM disease?
Plasmapheresis, glucocorticoids, cyclophosphamide
List the immune-complex small vessel vasculitides
- Anti-GBM
- IgA Vasculitis
- Cryoglobulinemia
Describe the pathologic findings associated with GPA
Granulomatosis with Polyangiitis (GPA)
- Necrotizign granulomas
- Few or no immune deposits
- Associated wtih c-ANCA/PR3+
Describe Polyarteritis Nodosa
- “Typical patient”:
- Arteries affected:
- Presentation:
Polyarteritis Nodosa
- “Typical patient”: Male 40-60 years old
- But can affect people of any age
- Arteries affected: Medium-sized muscular arteries, often at branch points
- Presentation:
-
Cutaneous
- Nodules, purpura, livedo, retucularis, ulcers
- Renal disease
- Neuropathy/Myalgia/Weakness
-
Mesenteric Ischemia
- “Stomach pain after large meals”
- Orchitis
-
Cutaneous
Describe Cryoglobulinemia
- “Typical Patient”:
- Arteries affected:
- Presentation:
Cryoglobulinemia
- “Typical Patient”: Person with MGUS, B-cell malignancy, chronic Hep C, Hep B, HIV, or autoimmune disease
- Arteries affected: Small vessels (immune complex mediated)
- Presentation:
- Digital ischemia
- Livedo reticularis and skin necrosis
What pathologic findings are associated with microscopic polyangiitis?
Necrotizing arteries
Few or no immune deposits
No granulomatous inflammation (will be present in GPA)
Does this statement apply to Giant Cell Arteritis, Takaysu Arteritis, or both?
Age of onset <40 years old
Takayasu Arteritis
Does this statement apply to Giant Cell Arteritis, Takaysu Arteritis, or both?
Affects large vessels
Both
Which vasculitis does this statement apply to?
Frequent URI
Granulomatosis with polyangiitis
How is GCA treated?
- High dose corticosteroids
- Tocilizumab (an anti IL-6 antibody)
Treat immediately (while waiting for biopsy) to prevent vision loss!
Which vasculitis does this statement apply to?
Most patients present with oral and genital ulcers
Behcets
Occurs most commonly in patients of Mediterranean, Middle Eastern, and Far Eastern descent
Describe the treatment for granulomatosis with polyangiitis and microscopic polyangiitis
Steroids
+ Rituximab or cyclophosphamide if severe
+ Methotrexate if not severe
How is GCA diagnosed?
- Labs
- Elevated ESR, CRP
- Thrombocytosis maybe
- Imaging
- Termporal artery ultrasound
- PET
- Biopsy
- Temporal artery
- Should be done urgently in all patients with suspected GCA
Which vasculitis is associated with chronic HepC infection?
Cryoglobulinemia type 2
Does this statement apply to Giant Cell Arteritis, Takaysu Arteritis, or both?
Constitutional symptoms
Both
Describe granulomatosis with polyangiitis
- “Typical patient”:
- Arteries affected:
- Presentation:
Granulomatosis with Polyangiitis (GPA)
- “Typical patient”: Caucasian 40-50 y/o or >70 y/o
- Arteries affected: Small to medium vessel vasculitis
- Presentation:
- Associated wtih c-ANCA/PR3+
-
Upper airway disease
- Saddle nose deformity, sinus nodules
-
Pulmonary disease
- Cavitary lung nodules
-
Glomerulonephritis
- Crescents
- Arthritis/arthralgia
- Neuropathy
- Ocular disease
-
Vascular
- Gangrene, venous thrombosis
-
Skin
- Oral ulcers, purpura, nodules
- Cardiac
- GI
What is the treatment for Kawasaki disease?
Why is it important to identify and treat early?
Aspirin + IVIG
Prevent heart disease/aneurysm later in life
Does this statement apply to Giant Cell Arteritis, Takaysu Arteritis, or both?
Bruits
Takayasu Arteritis
Describe Bechets Syndrome
- “Typical Patient”:
- Arteries affected:
- Presentation:
Bechets Syndrome
- “Typical Patient”: 20-30 year old person of Mediterranean, Middle Eastern, or Far Eastern descent
- Arteries affected: Any! Variable vasculitis
- Presentation:
- Oral ulcers
- Genital ulcers
- Other cutaneous lesions
- Ocular involvemnt
- Arthralgia
- Neurologic
- GI
- Inner ear
Describe the clinical presentation of Eosinophilic Granulomatosis with Polyangiitis (EGPA)
3 phases
- Prodrome
- Asthma, allegic rhinitis, nasal polyp
- Eosinophilic phase
- Tissue and periphery
- Vasculitic phase
- Cutaneous
- Cardiac
- Neurologic
- Renal
- Gastroenteritis
What are the three ANCA-associated vasculitides?
- Microscopic polyangitiis
- Granulomatosis with polyangiitis
- Eosinophilic granulomatosis with polyangiitis
Which vasculidities affect medium vessels?
Polyarteritis Nodosa
Kawasaki Disease
Describe giant cell arteritis
- “Typical patient”:
- Arteries affected:
- Presentation:
Giant Cell Arteritis
- “Typical patient”: Elderly Scandinavian woman
- Can also occur in men, but more common in women
- Arteries affected: Large vessels; cranial or extracranial involvement
- Presentation:
- New headache
- Constitutional symptoms
- Jaw claudication
- Partial or complete loss of vision
Which vasculitis does this statement apply to?
Presents most commonly in children <5
Kawasaki disease
What are the pathologic hallmarks of Kawasaki disease?
- Affects coronary arteries
- Destruction of luminal endothelial cells, elastic lamina, smooth muscle cells
- -> Arterial walll dilation +/- aneurysm formation
What complications are associated with Takayasu arteritis?
Aortic regurgitation and acute MI
Which arteritis is associated with jaw claudication, new headache, and partial or complete loss of vision?
Giant cell arteritis
Describe the treatment for Takayasu arteritis
- High-dose corticosteroids
- Methotrexate or Azathioprine
- Surgical bypass
- Angioplasty +/- stenting has poor outcomes
Which vasculitis does this statement apply to?
Associated with p-ANCA/MPO+
Microscopic polyangiitis
EGPA has p-ANCA/MPO in 40% of cases, but otherwise ANCA(-)
Which vasculitis does this statement apply to?
Treat with aspirin and IVIG
Kawasaki
Describe the pathologic findings associated with polyarteritis nodosa
- Transmural inflammation
- No giant cells or granulomas
- Common for different phases of vasculitis to occur within different vessels or one vessel at the same time
70 yo WM with chronic cough for past year. Exam shows nasal ulcers and lungs with diffuse crackles.
- Creat 4.5
- UA with 20 RBCs +casts
- c-ANCA +
- CXR with small scattered pulmonary nodules
- Lung bx shows vasculitis of small peripheral arteries and arterioles. +granulomatous inflammation adjacent to small arterioles
What is the most likely diagnosis?
Granulomatosis with polyangiitis
2nd peak in elderly patients, c-ANCA is the key
What is the most common form of systemic vasculitis in adults?
Giant cell arteritis
What is the appropriate management of a patient for whom you suspect giant cell arteritis?
Ugent temporal artery biopsy
Begin treatment with corticosteroids immediately
- Biopsy to confirm diagnosis, treat immediately to prevent complications
- Vision loss
Describe Takayasu Arteritis
- “Typical patient”:
- Arteries affected:
- Presentation:
Takayasu Arteritis
- “Typical patient”: Asian woman <40 years old
- Arteries affected: Aorta, subclavian, carotid most common
- Can affect any artery
- Presentation:
- Constitutional symptoms
- Unexplained lightheadedness
- Extremity pain/claudication
- Bruits
- Absent/diminshed pulses or BP difference >10 mmHg
What pathologic findings are associated with Eosinophilic Granulomatosis with Polyangiitis (EGPA)?
ANCA negative most of the tiem
Which vasculitis does this statement apply to?
Strawberry tongue
Kawasaki
Which vasculitis does this statement apply to?
p-ANCA +
Microcsopic polyangiitis
Does this statement apply to Giant Cell Arteritis, Takaysu Arteritis, or both?
Affects females > males
Both
GCA - F>M = 2:1
Takayasu - F>M = 9:1
Describe the pathological findings associated with Takayasu arthritis
- Granulomatous panarteritis
- Intimal hyperplasia
- Fixed stenosis and occlusions
- Occasional aneurysm
Which vasculitis typically presents in children, and is associated with acute onset fever, strawberry tongue, and rash?
Kawasaki Disease
Does this statement apply to Giant Cell Arteritis, Takaysu Arteritis, or both?
Age of onset >50 years old
Giant Cell Arteritis
Describe Anti-GBM disease
- “Typical Patient”:
- Arteries affected:
- Presentation:
Anti-GBM
- “Typical Patient”: Older children and adults
- Arteries affected: Small vessel (Immune-complex mediated)
- Presentation:
- Acute renal failure
- Alveolar hemorrhage
- No constitutional symptoms
Describe Kawasaki Disease
- “Typical patient”:
- Arteries affected:
- Presentation:
Kawasaki Disease
- “Typical patient”: Asian male child <5 years old
- M>F 2:1
- Arteries affected: Medium-sized muscular arteries, esp. coronary
- Presentation:
- Acute onset
- Fever > 5 days
- Rash (Basically anything except vesicular)
- Extremity edema
- Involvement of lips and mouth
Which vasculidities affect large vessels?
Takayasu Ateritis
Giant Cell Arteritis
What vasculitis is associated with MGUS or B cell malignancy?
Cryoglobulinemia Type 1
Describe the treatment for polyarteritis nodosa
- Mild
- Glucocorticoid monotherapy
- MTX or AZA if resistant
- Moderate
- Glucocorticoid + cyclophosphamide
- Viral-associated secondary PAN
- Check Hepatitis B
- Initiate appropriate antiviral therapy
Which vasculitis does this statement apply to?
c-ANCA +
Granulomatosis with Polyangiitis
Describe microscopic polyangiitis
- “Typical patient”:
- Arteries affected:
- Presentation:
Microscopic polyangiitis
- “Typical patient”: Middle age
- Arteries affected: Small vessels
- Presentation:
- Glomerulonephritis
- Pulmonary capillartis
- Peripheral neruopathy
- Skin lesions
- Weight loss
- Fever
- p-ANCA/MPO+
Which cytokine is associated with GCA?
IL-6
Treat with tocilizumab
Describe Eosinophilic Granulomatosis with Polyangiitis
- “Typical patient”:
- Arteries affected:
- Presentation:
Eosinophilic Granulomatosis with Polyangiitis
- “Typical patient”: Male 35-45 with new asthma
- Only slight male predominance
- Arteries affected: Small vessels
- Presentation:
-
Often ANCA negative
- p-ANCA/MPO+ in 40% of cases
- Eosinophilia/elevated IgE
- Adult onset asthma
-
3 phases
- Prodrome, Eosiniphilic, Vasculitic
-
Often ANCA negative
Which antigens are associated with p-ANCA and c-ANCA?
p-ANCA = Myeloperoxidase (MPO)
c-ANCA = Proteinase 3 (PR3)
