Studied by 9 people
What is Sarcoidosis characterized by?
noncaseating granulomas in 2 or more organs
What does Sarcoidosis affect 90% of the time?
Lungs
*followed by skin & eyes
What population is at a higher risk for sarcoidosis?
African-American, F>M, young & middle-age
What are the 4 stages of pulmonary manifestation of sarcoidosis?
1. Bilateral hilar adenopathy
2. Stage 1 + pulmonary infiltrates
3. Pulmonary infiltrates (no nodules)
4. Pulmonary fibrosis
What are the ocular manifestations of sarcoidosis?
Anterior uveitis
What are the derm manifestations of sarcoidosis?
Lupus pernio, Erythema nodosum
What are the MSK manifestation of sarcoidosis?
Oligoarthritis of lower extremities
What is the TRIAD of Lofgren's syndrome?
Acute arthritis, Erythema nodosum, Bilateral hilar adenopathy
What is Lofgren's syndrome?
Self-limiting, acute form of sarcoidosis
What lab will be elevated in a patient with sarcoidosis?
ACE
What is the definitive diagnostic study for sarcoidosis?
Tissue biopsy- Non-necrotizing non-caseating granulomas
What is the 1st line Tx for sarcoidosis?
Corticosteroids
What are 2nd and 3rd line tx for sarcoidosis?
2nd: MTX, hydroxychloroquine
3rd: -mabs
Who is Myositis is more common in?
Women, bimodal peak 5-15 yo & 40-60 yo
How does myositis typically begin?
Subacute onset over months
How does Myositis present?
Proximal muscle weakness, weak neck muscles (flexors), systemic sx, dysphagia, NO pain
What are sx of dermatomyositis?
Heliotrope rash, Gottron's papules, Sun-sensitive rash
What disease is a Shawl sign seen in?
Dermatomyositis (sun-sensitive rash)
What is Immune-mediated necrotizing myopathy?
Statin myopathy
What lab will be elevated in a patient with myositis/myopathy?
CPK-MM
What will be seen on an MRI in a patient with myositis/myopathy?
increase in signal intensity 2 involved muscles (quads)
Is ANA always present in polymyositis/dermatomyositis?
No, + in ~1/2 pts
What do Antisynthetase syndrome (anti-Jo-1 antibodies) manifest as?
idiopathic inflammatory myopathy, ILD, arthritis, Raynaud’s, fever, and/or mechanic's hands
Antisynthetase syndrome is associated with what antibodies?
Anti-Jo-1
What antibodies are associated with prior statin use causing an immune-mediated necrotizing myopathy?
Anti-HMGCR
What are Signal-recognition particle (SRP) antibodies associated with?
acute onset of severe weakness, inc incidence of cardiac involvement, and higher mortality rates
*seen in some pts w/ IMND
What is the definitive/gold standard diagnostic study for myositis?
Muscle biopsy
What will be seen on a muscle biopsy of a patient with polymyositis/dermatomyositis?
Perimysial lymphocytic infiltrate & perifasicular atrophy
What will be seen on a muscle biopsy of a patient with mitochondrial myositis?
Ragged red fibers
What is the 1st line tx for polymyositis/dermatomyositis?
Steroids
*followed by Azathioprine or MTX or cellcept
What is the 2nd line tx for severe/refractory polymyositis/dermatomyositis?
Cyclophosphamide, Rituximab, IVIG
Can MTX be used for myopathies with interstitial lung disease?
NO
What is the tx for IMNM (statin myopathy)?
stop statin, IVIG course if severe
Inclusion body myositis involves more of which muscles?
Distal
What antibody is present in Inclusion body myositis?
Anti-CN1A antibody
What will be seen on muscle biopsy of inclusion body myositis?
Rimmed vacuoles
What is the tx for inclusion body myositis?
No definitive tx → supportive (mild exercise & PT), poor prognosis
Which vasculitis is d/t intrinsic or autoimmune causes?
Primary
Which vasculitis is d/t infection, CA, or drugs?
Secondary
Classification of vasculitis is based on what?
size of blood vessel affected, biopsy findings of cellular involvement, & ANCA status
What conditions are mimickers of secondary vasculitis?
Atrial myxoma, Cholesterol emboli syndrome (post-angiogram), Infectious endocarditis
What are the 2 forms of large vessel vasculitis?
Takayasu's arteritis & Giant cell arteritis
What is “Pulseless disease”?
Takayasu's arteritis
What is Takayasu's arteritis?
rare, systemic large-vessel granulomatous inflammation of the aorta and its major branches (subclavian)
Takayasu's arteritis mostly affects what population?
Asian females ~30 yo
What are the most common sx of Takayasu's arteritis?
HTN, carotid bruits, upper extremity BP discrepancies & pulses, arm claudication
What is the diagnostic study for Takayasu's arteritis?
Angiography of the aorta and its branches
What is the tx for Takayasu's arteritis?
Steroids
What is temporal arteritis or cranial arteritis?
Giant cell arteritis
What artery is most commonly affected in giant cell arteritis?
Temporal artery
50% of the time Giant cell arteritis is associated with what other disease?
Polymyalgia Rheumatica (PMR)
Who does Giant cell arteritis affect?
50-60 yo, W>M
Giant cell arteritis usually presents with UNIlateral sx such as?
HA (temporal), scalp tenderness, jaw claudication, monocular visual loss (amaurosis fugax), aortic arch syndrome -arm claudication
What is the definitive diagnostic study for Giant cell arteritis?
Temporal artery biopsy- multinucleated giant cells & lymphocytic granulomas
What is the 1st line tx for Giant cell arteritis?
High dose steroids
When should a biopsy to confirm giant cell arteritis be done?
Within 1 week of starting steroids
Why do you treat giant cell arteritis with steroids right away?
Avoid visual loss
After a temporal artery biopsy, what med should be started?
ASA 81mg
What biologic is now approved by the FDA for treatment of Giant cell arteritis?
IL-6 inhibitor: Tocilizumab (Acterma)
What is Polymyalgia rheumatica (PMR)?
Widespread aching and stiffness of limb-girdle muscles in older adults
What are the sx of PMR?
limb girdle pain, aching & stiffness, worst on arising in the morning, muscle strength preserved
Is Polymyalgia Rheumatica (PMR) a myopathy?
No, bc muscle strength is preserved
Who does PMR affect most?
> 50 yo (usually 70-80), W > M, whites >>
What can be used as a diagnostic response for Polymyalgia rheumatica (PMR)?
Low dose steroids improves/resolves symptoms within 24-48 hours
What is the Gold standard diagnostic study for Polymyalgia rheumatica (PMR)?
ESR or CRP (significantly elevated)
What is the 1st line tx for Polymyalgia rheumatica (PMR)?
Low dose steroids (prednisone)
What population is most commonly affected by Kawasaki's disease?
Asian kids
What is a major complication of Kawasaki's disease?
Coronary artery aneurysms or thrombosis
"FEBRILE" mnemonic for symptoms of Kawasaki's disease:
F- Fever for at least 5 days
E- Enanthem: strawberry tongue
B- Bulbar conjunctivitis
R- Rash
I- Internal organ involvement (GI, heart, liver)
L- Lymphadenopathy
E- Erythematous Extremities (palms & soles)
What is the tx for Kawasaki?
baby ASA & IVIG
What are the 3 types of ANCA-Associated Vasculitis (+AAV)?
GPA, EGPA, MPA
ANCAs directed to proteinase 3 (PR3) receptor/C-ANCA = ______
GPA
ANCAs directed to myeloperoxidase (MPO) receptor/P-ANCA = ______
EGPA or MPA
What is the triad of GPA?
URI, Pulmonary, and Renal involvement
What are the URI manifestations of GPA?
Sinusitis, Nasal/Oral ulcerations, "Saddle-nose" deformities
What are the derm manifestations of GPA?
Nodules, palpable purpura, ulcerations
What is the diagnostic lab test (90% specificity) for GPA?
+ C-ANCA
What pulmonary manifestations of GPA are seen on CXR?
nodules & infiltrates with/without cavitary granulomas
What are renal manifestation of GPA?
Necrotizing glomerulonephritis- RBC casts
What is the 1st line tx for GPA/EGPA/Microscopic polyangitis?
Steroids, MTX, Azathiprine/MMF
What are key features of EGPA?
History of asthma and/or allergic rhinitis & peripheral eosinophilia
What are the sx of EGPA?
atopic, asthmatic, skin & lung nodules (non-cavitary), peripheral neuropathy, eosinophilia
What are the diagnostic studies for EGPA?
Eosinophils on CBC & Positive anti-MPO or P-ANCA
What is the tx for EGPA?
steroids, immunosuppressive, IL-5 inhibitors (Mepolizumab)
What is small vessel capillaritis?
Microscopic polyangitis/periarteritis
Microscopic polyangitis is small vessel involvement resulting in what?
Rapidly progressive glomerulonephritis (RPGN) & Pulmonary alveolar capillaritis
*also affects heart, palpable purpura common
What lab is very common in Microscopic polyangitis?
(+) p ANCA
What is the tx for Microscopic polyangitis?
steroids 1st, then MTX or Azathioprine
What organs are usually spared in Polyarteritis Nodosa (PAN)?
Lungs
Polyarteritis Nodosa (PAN) is strongly associated with what disease?
Hep B, Hep C, and HIV
How does PAN present?
painful skin ulcerations, extremity gangrene, bowel infarction, hepatic infraction, renal involvement, mononeuropathy multiplex (sural nerve/foot drop), parasthesia
What are the derm manifestation of Polyarteritis Nodosa (PAN)?
Palpable purpura -MC, splinter hemorrhages, nodules, livedo reticularis
What should you always check for in Polyarteritis Nodosa (PAN)?
Hep B, Hep C, and HIV
Where will male patients commonly experience pain/tenderness with Polyarteritis Nodosa (PAN)?
Testicular pain/tenderness
Are ANCAs are always present in Polyarteritis Nodosa (PAN)?
No -rarely present
What would you see on an angiogram in Polyarteritis Nodosa (PAN)?
Multiple aneurysms — Beading or Strings of pearls
What is the tx for PAN?
steroids — IV pulse & cyclophosphamide
*survival is not high, poor prognosis
What is Leukocytoclastic vasculitis characterized by?
Palpable purpura, especially of the legs
What is definitive diagnostic test for LCV?
skin biopsy
What is the tx for LCV?
address underlying cause
Note 
Flashcard (58)