Vasculitis and Fibromyalgia Flashcards
What is Takayasu’s arteritis? Where in the body does it usually occur, and whom does it usually affect?
- Large vessel granulomatous vasculitis with massive intimal fibrosis and vascular narrowing.
- Aorta
- Young females (15-30 yo)
What are some of the classic s/sx of Takayasu’s arteritis?
- Decreased to absent peripheral pulses
- B symptoms during inflammatory phase
What are the two major phases of Takayasu’s arteritis?
Inflammatory phase
Pulseless phase
What are some major sequelae of Takayasu’s arteritis?
- Renovascular HTN
- Ocular involvement
What is the characteristic type of inflammation of the vessels in Takayasu’s arteritis?
Segmental and patchy granulomatous inflammation of the aorta or its branches
What is the haplotype that is associated with Takayasu’s arteritis?
HLA-B52
What are the major criteria for Takayasu’s arteritis? (5)
- Age less than 40
- Claudication of extremities
- Decreased brachial artery pulse
- BP difference of more than 10 mmHg between arms
- Bruit over subclavian or aorta
What is the gold standard test for Takayasu’s arteritis?
Arteriogram showing occlusion or narrowing in the aorta or its main branches
Takayasu’s arteritis must occur before what age to meet its diagnostic criteria?
40
What is the BP difference between arms that is needed in the diagnostic criteria for Takayasu’s arteritis?
more than 10 mmHg between arms
What is the gold standard for diagnosing vasculitis?
Bx
Who does Kawasaki’s disease affect?
Asian children less than 4 yo
What are the s/sx of Kawasaki’s disease? (5)
- Marked Fever
- LAD /peripheral edema
- conjunctival injection
- Strawberry tongue
- Desquamating rash
What is the treatment fro Kawasaki’s disease?
ASA and IVIG
What are the s/sx of Churg-Strauss syndrome?
- Asthma
- Sinusitis
- Palpable purpura
- Peripheral neuropathy
What are the labs that are elevated with Churg-Strauss syndrome? (2)
- IgE
- MPO-ANCA (p-ANCA)
What are the s/sx of granulomatosis with polyangiitis (Wegener’s syndrome)?
- URI
- Perforation of nasal septum
- Chronic sinusitis
- OM
- Hematuria with red cell casts
What is the classic triad for granulomatosis with polyangiitis (Wegener’s syndrome)?
- Focal necrotizing vasculitis
- Necrotizing granulomas in the lung and upper airway
- Necrotizing glomerulonephritis
- Focal necrotizing vasculitis
- Necrotizing granulomas in the lung and upper airway
- Necrotizing glomerulonephritis = ?
granulomatosis with polyangiitis (Wegener’s syndrome)
What are the abs that are elevated with granulomatosis with polyangiitis (Wegener’s syndrome)?
PR3-ANCA (c-ANCA)
What is the treatment for granulomatosis with polyangiitis (Wegener’s syndrome)?
Cyclophosphamide
What are the two major vasculitides that affect large arteries?
- Giant cell (temporal) arteritis
- Takayasu arteritis
Small or small-medium vasculitis: HSP
Small
Small or small-medium vasculitis: SLE
Medium
Small or small-medium vasculitis: cryoglobulinemia
Small
Small or small-medium vasculitis: polyarteritis nodosa
Medium
Small or small-medium vasculitis: Wegener’s
Medium
Small or small-medium vasculitis: rheumatoid vasculitis
Medium
Small or small-medium vasculitis: Churg-Strauss
Medium
Small or small-medium vasculitis: microscopic polyangiitis
Medium
Small or small-medium vasculitis: Kawasaki
Medium
Small or small-medium vasculitis: vasculitis associated with malignancy
Small
Polyarteritis Nodosa is associated with what infectious disease?
Hep B
What age group Polyarteritis Nodosa classically affect?
Young adults
What are the s/sx of Polyarteritis Nodosa? (5)
- B symptoms
- HTN
- Abdominal pain / melena
- Renal damage
- Neurological disease
What vessels does Polyarteritis Nodosa classically involve?
renal and visceral vessels (not pulmonary arteries)
What type of hypersensitivity rxn is Polyarteritis Nodosa?
Type III
What are the characteristics bx findings of Polyarteritis Nodosa?
Transmural inflammation of the arterial wall with fibrinoid necrosis
What are the classic arteriogram findings of Polyarteritis Nodosa?
Innumerable microaneurysms and spasms
What is the treatment for Polyarteritis Nodosa? (2)
Corticosteroids
Cyclophosphamide
Who does Buerger’s disease (thromboangiitis obliterans) classically affect?
heavy male smokers
What are the bx findings of Buerger’s disease (thromboangiitis obliterans)?
Segmental thrombosing vasculitis
What is the treatment for Buerger’s disease (thromboangiitis obliterans)?
Smoking cessation
What are the s/sx of Buerger’s disease (thromboangiitis obliterans)? (2)
- Intermittent claudication leading to gangrene / amputation of digits
- Raynaud’s syndrome
What are some of the general s/sx that may lead you to suspect a vasculitis?
- Multisystem dz
- Skin lesions
- Renal involvement
- B symptoms
What is mononeuritis multiplex?
Death of vasavesorum of nerves lead to nerve deficits
What diseases /drugs need to be r/o prior to diagnosing vasculitis?
- Thrombotic disease
- Antiphospholipid syndrome
- Cocaine / amphetamines
Which classically involves the lungs: PAN or MPA?
MPA
Which is ANCA positive: PAN or MPA?
MPA
What are the organs that are commonly involved in MPA?
Lungs
Kidneys
skin (palpable purpura)
True or false: there are no granulomas with MPA
True
What is the classic angiogram findings of polyarteritis nodosa?
String of pearls
What are the four criteria for diagnosing HSP?
- Palpable purpura
- Less than 20 years
- Bowel angina
- Granulocytes in walls of arterioles or venules
HSP occur below what age (according to the classification criteria)?
20
What is the classic triad of symptoms for HSP?
- Palpable purpura
- Arthralgias
- Abdominal pain/ melena
What is the ab that is deposited with HSP?
IgA
What happens to the severity of HSP as kids age?
Worse–need to monitor teenagers
HSP classically occurs after what?
A viral infection
What is cryoglobulinemia? What are the clinical features of it (3)?
- Immunoglobulins that undergo reversible precipitation in the cold
- Acral cyanosis, ulcerations/ necrosis, raynaud’s phenomenon
What are the clinical features of cryoglobulinemia that are related to immune complex deposition? (4)
- Purpura
- Arthralgias
- Nephritis
- Neuropathy
What are the 4 diagnostic criteria for Wegener’s granulomatosis (GPA)?
- Nasal or oral inflammation
- Abnormal chest radiograph (nodules / cavities)
- Urinary sediment
- Granulomatous inflammation on bx
What are the CXR findings of GPA?
Nodules
cavities
What are the UA findings of GPA?
Sediment with more than 5 RBCs / hpf or RBC casts
What are the nasal / oral mucosal findings of GPA?
Nasal or oral inflammation, causing a nasal deformity
What is polymyalgia rheumatica?
a syndrome with pain or stiffness, usually in the neck, shoulders, upper arms, and hips, but which may occur all over the body. The pain can be very sudden, or can occur gradually over a period. Most people with PMR wake up in the morning with pain in their muscles; however, cases have occurred in which the person has developed the pain during the evenings or has pain and stiffness all day long.
What are the symptoms of polymyalgia rheumatica?
- Pain and stiffness in the shoulders and hips, often with B symptoms
- Morning stiffness
Does polymyalgia rheumatica cause muscular weakness?
No
What is ocular findings is associated with polymyalgia rheumatica?
Temporal arteritis
What are the ESR, CRP, and CK findings of polymyalgia rheumatica?
Elevated ESR and CRP
Normal CK
Who is classically affected with polymyalgia rheumatica?
Women over 50 years, usually of northern european descent
What is the treatment /prognosis for polymyalgia rheumatica?
Rapid response to low doses of corticosteroids
What are the classic s/sx of giant cell arteritis?
- Jaw Claudication
- HA
- Temporal artery abnormality
- Visual loss
What are the three labs that are classically elevated with giant cell arteritis?
- CRP
- ESR
- Platelets
What is the treatment for giant cell arteritis?
Steroids
What is the severe cardiac sequelae of giant cell arteritis?
Aortic dissection
What are the histological findings of giant cell arteritis?
Focal granulomatous inflammation of the vessels with giant cells
What is the most commonly affected vessel with giant cell arteritis?
Branches of Carotids
What, generally, is fibromyalgia?
Chronic, widespread musculoskeletal pain associated with stiffness, paresthesias, and poor sleep
What is the treatment for fibromyalgia? (3)
- Exercise
- SNRIs
- anticonvulsants
Having a first degree relative with fibromyalgia imparts a (__)x greater risk of having it.
8.5x
What is the proposed pathogenesis of Fibromyalgia?
Deficits in GABAergic and glutamatergic mechanisms
What are the associated diseases with FM?
- IBS
- Interstitial cystitis
What are some of the common complaints of FM patients?
- Multiple allergies
- Palpitations
- Dysmenorrhea
- Orthostatic intolerance
When should SLE be screened for with fibromyalgia?
Only if history and physical suggests inflammatory systemic rheumatic disease
What is the ROS like with fibromyalgia?
Positive everywhere
What are the endocrine disorders that may have similar s/sx of FM?
- Hypothyroidism
- Hyperparathyroidism
- Cushing’s
- Vit D deficiency
Do patients feel better immediately after stopping a statin with statin-induced myopathy?
No–2-3 months
What should be done with a new diagnosis of FM?
Just like somatic disorder:
- Reassure no infx or other disease
- Stress and mood management
What are the two treatments for restless leg syndrome? MOA?
- Ropinirole -dopamine agonist
- Pramipexole-dopamine agonist
What is the MOA of bupropion?
Dopamine reuptake inhibitor
What are the three medications that should NOT be used with fibromyalgia?
- Narcotics
- NSAIDs
- Steroids
What is the MOA of duloxetine?
SNRI