7.1 Vasculitis Flashcards
60 y female presents with headache, fatigue, muscle and joint aches, unilateral vision loss, jaw claudication, fever, temporal tenderness. Explain the disease. What labs, and other studies are needed for diagnosis? What is the treatment?
Likely giant cell arteritis caused by inflammation of the walls of the vessels branching from the carotid arteries. Inflammation can lead to occlusion causing ischemia to eye and scalp leading to pain and blindness. Inflammation leads to generalized symptoms.
Labs: ESR, CBC
Dx: biopsy needed, lesions are segmental, looking for inflammation in media and inflammatory cells in the adventitia including lymphs. US may show occlusion or stenosis of temporal or occipital arteries
Tx: Prednisone as soon as Dx is suspected, use for 1-2 months and then a taper, ASA to prevent thrombosis and stroke, vit D and Ca to counter the long term use of steroids and their effects on bones, high risk of blindness without treatment
40 y asian female presents with vertigo and syncope. She also c/o fever, malaise, fatigue. Physical exam shows decreased pulses in carotid and UE. Likely Dx? Workup? Tx?
Takayasu Arteritis: inflammation of the aortic arch and branches
Labs: ESR, biopsy shows inflammatory cells in vessel wall with giant cells and vascular fibrosis
Image: arteiography may show stenosis, CT or MRI can show vessel wall abnormalities
Treatment: corticosteroids, immunosuppression, bypass grafting of vessels
Haw are temporal arteritis and takayasu arteritis the same and different?
Same: large vessel inflammation, giant cells and other cells in wall on biopsy, generalized symptoms from inflammation, treated with steroids
Different: Temporal has vision effects, scalp and temporal tenderness, is in women over 50
Takayasu decreases pulses, is in asian women under 50, can cause MI and CVA
Which vasculitis is associated with HBsAg?
Polyarteritis nodosa
Which vasculitis can kill the patient if untreated?
Polyarteritis nodosa
Young male patient presents with abdominal pain, HTN, melena, hematuria, anemia, neuropathy, palpable purpura, joint pain, and skin ulcers. Dx? Disease process? Biopsy findings? Epidemiology? Organs affected? Workup? Treatment?
Polyarteritis Nodosa
Necrotizing vasculitis leading to ischemia involving many organs but sparing the lungs. Ischemia causes HTN, hematuria, abdominal pain with melena, neurologic disturbances, and skin lesions.
Commonly affects young more than old and men more than women.
Labs: increased WBC, anemia, ESR, proteinuria, hematuria, negative p-ANCA
Angiography would show aneurisms
Biopsy can be helpful, healing with fibrosis will show string of pearls on histology
Treatment: steroids and immunosuppression, fatal if not treated
What vasculitis is more common in men than women?
Polyarteritis nodosa
What disease affects kids less than 4 years old? What nationality is most common?
Kawasaki disease
Often asian kids
3 y asian kid presents with fever conjunctivitis, red palms and soles with eventual desquamation, and large cervical lymph nodes. Dx? Biggest concerns? Workup? Treatment?
Kawasaki Disease: necrotizing vasculitis involving vessels of all sizes. Can involve coronary arteries leading to aneurysm, MI, death.
Labs: can show autoantibodies to endothelial cells
Radiology: echocardiogram shows coronary aneurysms and is most useful when used with dobutamine stress test; angiography can show vessel irregularities
Treatment: ASA to prevent thrombosis, IV gamma globulin, frequently self-limited
What disease causes autoamputation of the digits and is associated with Raynaud’s? What is the treatment?
Buerger Disease: necrotizing vasculitis of the digits associated with heavy smoking. Treatment is smoking cessation.
Describe temporal cell arteritis
Granulomatous vasculitis involving branches of the carotid artery
Common in females over 50. Most common arteritis
Presents with headache, visual changes, jaw claudication, flu-like sx of joint and muscle pain (polymyalgia rheumatica), increased ESR
Biopsy shows giant cells and inflammation
Treatment with steroids to prevent blindness
Describe takayasu arteritis
Granulomatous vasculitis involving aortic arch and branches
Asian females less than 50
Presents with visual and neurological symptoms and weak or absent pulses in UE and decreased carotid pulse, ESR elevated
Treat with steroids
Describe polyarteritis nodosa
Necrotizing vasculitis leading to ischemia that affects most organs except the lungs
Most common in young adults and males over females
Presents with HTN, melena, abdominal pain, hematuria. neuropathy, skin lesions–palpable purpura and ulcers of varying ages.
String of pearls on biopsy
Treat with steroids and cyclophosphamide (chemo agent that decreases immune system), fatal if not treated
Describe Kawasaki disease
Asian kids less than 4 years old
Fever, conjunctivitis, maculopapular rash on palms and soles that eventually desquamates, cervical lymph nodes
Can involve coronary arteries leading to aneurysms, MI, and sudden death
Treat with ASA and IVIG, disease is usually self-limited
Describe Buerger disease
Necrotizing vasculitis involving the digits
Caused by heavy smoking and therefore treated with smoking cessation
Causes ulceration, gangrene and autoamputation of the fingers and toes
Middle-aged male presents with hemoptysis and sinusitis and hematuria. Likely Dx? Disease process? Labs? Biopsy? Treatment?
Wegener Granulomatosis
Necrotizing vasculitis involving the the nasopharynx, lungs, and kidneys
Classically presents in middle-aged males with sinusitis, nasopharyngeal ulceration, hemoptysis with bilateral nodular lung infiltrates, and hematuria due to rapidly progressive glomerulonephritis
Labs: c-ANCA correlated with disease activity
Biopsy: necrotizing granulomas with adjacent vasculitis
Treatment: cyclophosphamide and steroids, relapses are common
What vasculitis is associated with c-ANCA?
Wegener Granulomatosis
Young male presents with hemoptysis and hematuria and fever and malaise. Examination of the nasal mucosa shows no signs of sinusitis or ulcers. Biopsy of vessels lacks any sign of granulomas. Dx? Disease process? Labs? Treatment?
Microscopic Polyangiitis
Necrotizing vasculitis of multiple organs, but especially the lungs and kidneys
Presents similar to wegener granulomatosis except for the lack of nasopharyngeal involvement and granulomas
Labs: p-ANCA correlates with disease activity
Treatment: steroids and cyclophosphamide, relapses common
Which two vasculitides present very similar and are associated with p&c-ANCA? How are they different in presentation?
Wegener Granulomatosis: c-ANCA and nasopharyngeal involvement
Microscopic Polyangiitis: p-ANCA and no nasopharyngeal involvement
Describe Churg-Stauss Syndrome
Necrotizing granulomatous inflammation with eosinophils involving multiple organs but especially the lungs and heart
Asthma and peripheral eosinophilia are common
Serum p-ANCA correlate with disease activity
Treatment is steroids and immunosuppressive therapy
What vasculitis is associated with asthma? What organs are primarily involved?
Eosinophilic granulomatosis with polyangiitis
Churg-Strauss Syndrome
What two vasculitides are correlated with p-ANCA, and which with c-ANCA?
p-ANCA: Churg-Strauss and Microscopic polyangiitis
c-ANCA: Wegener granulomatosis
Young patient presents few weeks after URI with palpable purpura on buttocks and legs, GI pain, bleeding, hematuria. Dx? Cause? Treatment?
Henoch-Schonlein Purpura
Vasculitis due to IgA immune complex deposition in skin and kidneys leading to IgA nephropathy
Treatment is steroids if needed, usually self-limited
What is the most common vasculitis in kids?
Henoch-Schonlein Purpura
Describe the challenges of biopsy in regard to temporal arteritis
Lesions are intermittent and therefore a long piece of the artery must be taken and the entire length must be examined. It also means that a negative result does not rule out the disease.
What vasculitis is also called pulseless disease?
Takayasu arteritis
Which vasculitis involves all organs except the lungs?
Polyarteritis nodosa
Which of the vasculitides has skin lesions?
Polyarteritis nodosa
Which of the vasculitides involves the nasopharynx?
Wegener granulomatosis
Which vasculitide is caused by IgA immune complex deposits in the skin and kidneys?
Henoch-Schonlein Purpura
You suspect a vasculitis in a patient and you run an ANCA study and find it positive for p-ANCA. What features will help to distinguish the two diseases that are possible?
Features of asthma and eosinophilia and granulomas all with Churg-Strauss, but absent with Microscopic polyangiitis
Which small vessel vasculitide affects primarily the lungs and the heart?
Churg-Strauss Syndrome
