Rheumatology

Question 1

TNF adverse effects to know

Answer 1

1) hold in severe infections
2) reactivation of hepatitis B, which can be fatal
3) check for latent TB and screen regularly
4) avoid in class III/IV heart failure
5) Considered safe in pregnancy
6) positive ANA, with lupus-like syndromes
7) demyelination, similar to MS

Warning for lymphoma risk, though this is controversial

Question 2

anti-smith

Answer 2

SLE (specific for diagnosis)

- do not correlate with disease activity, use to diagnose

Question 3

anti-U1-RNP

Answer 3

MCTD

Question 4

Anticentromere pattern of ANA

Answer 4

CREST syndrome, SSc and PH

Question 5

anti-dsDNA ab

Answer 5

correlates with SLE disease activity

Question 6

anti-smooth muscle

Answer 6

autoimmune hepatitis

Question 7

anti-La/SSB

Answer 7

Sjogren syndrome, neonatal lupus

Question 8

anti-Scl70

Answer 8

SSc, pulmonary fibrosis, diffuse cutaneous SSc

Question 9

Antihistone

Answer 9

drug-induced SLE

Question 10

Anti-Ro/SSA

Answer 10

Sjogren syndrome, subacute cutaneous lupus

Question 11

c-ANCA (anti-PR3)

Answer 11

GPA

Need a GPA of 3 to Pass Rheumatology (PR3)

Question 12

p-ANCA (anti-MPO)

Answer 12

Eosinophili granulomatosis with polyangiitis, and MPA

Question 13

Anti-Jo-1

Answer 13

Polymyositis, and antisynthetase syndrome

Question 14

Anti-CCP

Answer 14

RA

Question 15

Anti-TNF biologics used in RA

Answer 15

1) Enteracept
2) Adalimumab
3) infliximab
4) Golimumab
5) Certolizumab

increased risk of infection, avoid live vaccines

Question 16

Non-TNF biologics used in RA

Answer 16

1) Tocilizumab/sorilumab (Anti- IL-6)
2) Abtacept (CTLA-4 IgG)
3) Ritixuimab (B cell agent)
4) Anakinra (IL-1 inhibitor)

Question 17

Findings in microscopic polyangiitis

Answer 17

nongranulomatous necrotizing pauci-immune vasculitis of the small vessels or pauci-immune necrotizing crescenteric GN

Clinical findings: pulmonary hemorrhage, pauci-immune small-vessel necrotizing vasculitis of the skin, proteinuria, inflammatory arthritis, positive p-ANCA (MPO, perinuclear)

Question 18

histology differences between GPA, MPA, Eosinophilic GPA

Answer 18

GPA: granulomatous pauci-immune vasculitis
MPA: nongranulomatous pauci-immune vasculitis
E GPA: granulomatous pauci-immune vasculitis with eosinophilia

Question 19

In severe refractory tophaceous gout, which IV therapy is indicated

Answer 19

Pegloticase infusion (porcine-derived uricase enzyme)

Stop other uric acid treatments, as serum urate levels are trended to screen for antibody development, and this may obscure this screening

Start prophylaxis for gout flares with either NSAIDs, glucocorticoids, or colchicine

Question 20

Is the presence of HLA-B27 a diagnostic criterion for ankylosing spondylitis?

Answer 20

NO

It may be helpful with clinical presentation c/w ankylosing spondylitis, but without classic imaging findings

Diagnostic criteria for Ankylosing Spondylitis

• younger than 45
• inflammatory back pain >3 months duration
• bilateral sacroiliitis on imaging

Question 21

What GI finding is seen in diffuse cutaneous systemic sclerosis?

Answer 21

GAVE: Gastric Antral Vascular Ectasia

• proliferation of blood vessels typically in the antrum, appears like watermelon stripes
• treat with APC or laser coagulation

Question 22

What agent can be used off label for refractory gout?

Answer 22

Anakinra/canakinumab (IL-1 inhibitors)

Question 23

Antibodies associated with neuropsychiatric SLE disease

Answer 23

1) antineuronal
2) anti-NMDA receptor
3) antiribosomal P
4) antiphospholipid
5) lupus anticoagulant

Question 24

Drug-induced lupus (antihistone Ab +), culprit drugs

Answer 24

1) Hydralazine
2) Provainamide
3) INH
4) Minocycline
5) TNF-alpha inhibitors

Question 25

Drug-induced cutaneous lupus, culprit drugs

Answer 25

1) ACE inhibitors 2) HCTZ 3) Hydralazine 4) Griseofulvin 5) NSAIDs 6) PPIs 7) Terbinafine 8) TNF-alpha inhibitors

Question 26

Triad that makes up Felty Syndrome

Answer 26

1) Rheumatoid arthritis (usually aggressive, long-standing) 2) Neutropenia 3) Splenomegaly Other clinical findings: - Mononeuritis multiplex - Necrotizing skin infections - pleuropericarditis - lymphadenopathy - episcleritis Associated risk for: - severe infections - lower extremity ulcers - lymphoma - vasculitis

Question 27

Causes of acquired PAN

Answer 27

1) HBV 2) HIV 3) Hairy cell leukemia

Question 28

Inclusion body myositis findings

Answer 28

An inflammatory myopathy involving the proximal and distal muscles - typically symmetric (though muscle redistribution may be asymmetric) - usually >50 years old - Serum CK: 10-12x ULN - insidious in onset (different from inflammatory myopathies) - EMG: myopathic, neurogenic or mixed changes - muscle biopsy: muscle fibers with multiple rimmed vacuoles

Question 29

When to consider systemic therapy for Sjogren syndrome

Answer 29

1) Evidence of joint disease (erosions, bone damage) 2) CNS or systemic disease Consider methotrexate for arthritis Cyclosporine/rituximab for severe or systemic CNS disease

Question 30

Findings in scleritis

Answer 30

1) eye pain 2) pain with gentle palpation of the globe 3) photophobia 4) scleromalacia (thinning of the sclera -> dark area), may lead to perforation Rheumatoid arthritis is a common inflammatory cause Note that episcleritis is infrequently associated with inflammatory/rheumatological disease

Question 31

What makes up the arthritis-dermatitis syndrome of disseminated gonococcal infection?

Answer 31

1) Tenosynovitis 2) Dermatitis (painless pustular or vesiculopustular lesions) 3) polyarthralgia without frank athritis Also will see fever, chills and malaise

Question 32

Lofgren syndrome (seen in acute sarcoid arthritis)

Answer 32

1) hilar adenopathy 2) Acute arthritis 3) Erythema nodosum The arthritis is usually oligoarticular

Question 33

Takayasu arteritis vs. subcranial GCA

Answer 33

TA is seen in young women (9:1 ratio) between 15 and 24. Affects the aorta and major branches. Subcranial GCA usually has preceding symptoms c/w PMR, as well as vasculitis affecting the large subcranial vessel. Seen in elderly patients and is a late feature of GCA. May cause heart failure.

Question 34

Secondary causes of Calcium Pyrophosphate (CPP) arthritis (pseudogout)

Answer 34

1) hyperparathyroidism 2) Hypothyroidism 3) hypophosphatemia 4) hypomagnesemia 5) hemochromatosis

Question 35

In GPA relapse, what medication is used to introduce remission

Answer 35

Rituximab Shown to be superior to cyclophosphamide for relapsed ANCA vasculitis

Question 36

Treatment for primary angiitis of CNS (PACNS)

Answer 36

Cyclophosphamide and high dose glucocorticoids

Question 37

What are the features of Adult-Onset Stills Disease?

Answer 37

1) Spiking fevers 2) Pharyngitis 3) Evanescent salmon pink rash 4) Adenopathy, HSM 5) Elevated liver enzymes 6) leukocytosis with neutrophil predominance 7) inflammatory arthritis 8) Myalgias 9) extremely high ferritin Look for this during pregnancy or in postpartum period

Question 38

Anti-HMG Co-A reductase antibody

Answer 38

Seen in immune-mediated nectorizing myopathy - consider in patient on a statin presenting with persistent myopathy following discontinuation of statin - also use muscle biopsy to aid in diagnosis

Question 39

Anti-cytosolic 5'-nucleotidase 1A antibodies

Answer 39

seen in inclusion body myositis (high specificity)

Question 40

Treatment for undifferentiated connective tissue disease (UCTD)

Answer 40

Hydroxychloroquine

Question 41

Recommended treatments for SLE nephritis

Answer 41

1) Mycophenolate mofetil (class V, if no renal dysfunction) 2) Cyclophosphamide (not preferred d/t SE) 3) Azathioprine 4) Rituximab Mycophenolate mofetil is teratogenic, must be stopped 3 months prior to anticipated pregnancy

Question 42

Side effects of DMARD therapy in RA

Answer 42

Methotrexate: hepatitis, bone marrow suppression Leflunomide: GI upset, diarrhea, ALT elevation, cytopenias, infection, teratogenesis, and axonal neuropathy RA patient recently started on leflunomide now with neuropathy symptoms -> stop the leflunomide

Question 43

What class of drug is beliumumab? What is it used for?

Answer 43

B-lymphocyte stimulator (BLyS) protein inhibitor Useful in mild to moderate SLE on glucocorticoids, especially in presence of mucocutaneous or joint symptoms .

Question 44

What are the features of colchicine toxicity?

Answer 44

1) Rhabdomyolysis 2) AKI 3) Pancytopenia Colchicine should be avoided in conjunction with CYP3A4 inhibitors (e.g. clarithromycin, fluconazole)

Question 45

Skin findings in subacute cutaneous lupus

Answer 45

Annular with central clearing or papulosquamous with patchy erythematous plaques and papules. ~ 50% of patients with SCLE also have SLE

Question 46

Drug-induced ANCA vasculitis. What drugs are implicated?

Answer 46

1) Hydralazine 2) Minocycline 3) PTU, Methimazole, Carbimazole (antithyroid drugs) Look for double positive titers

Question 47

Longstanding Systemic Sclerosis is a risk factor for what?

Answer 47

NHL (B-cell lymphoma) Particularly if disease present for >6 years

Question 48

How to differentiate between primary and secondary Raynaud's on exam?

Answer 48

Nail fold capillary examination (normal in primary RP)

Question 49

Signs of methotrexate toxicity

Answer 49

Somatitis Megaloblastic anemia Depletes folic acid stores, make sure folic acid is supplemented for all patients taking MTX