Rheum E2- Sarcoidosis, Inflammatory Myositis, & Vasculitides

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