Rheum HY

Question 1

Pt presents with:
HTN (200s/100s)
Low Platelets ( <50k)
Cr 3 elevated!
and has h/o Scleroderma (affects entire length of limb)
Dx/Treatment?!

Answer 1

Scleroderma renal crisis
Ace-I
(Lisinopril)

Question 2

Nail pitting,
digital ischemia in cold weather (raynouads),
chronic heartburn (esophageal dysmotility).

Dx?
MCCOD?

Answer 2

CREST Scleroderma
Pulm HTN

(aka Limited Cutaneous Scleroderma)

Vasculopathy → pulmonary arteries lumen becomes fibrosed & narrowed.

Question 3

Classic rheumatologic drug to avoid with scleroderma

Answer 3

Steroids
(increase risk of renal crisis)

Question 4

Order of pulmonary manifestation of diffuse scleroderma

Answer 4

1st: Interstitial lung disease (fine crackles)
2nd: Pulm HTN

(s/t alveolar basement membrane fibrosis)

Question 5

Hand ± wrist joint pain/swelling improves with use
Nail Pitting
Sausage digits (fingers or feet)
Dx?

Answer 5

psoriatic arthritis

Question 6

40-50s Female
Finger (MCP/PIP) pain + Stiffness better w/ movement
Bilateral wrist/hand joint pain/swelling
No skin findings
↑ ESR
(–) Rheumatoid Factor
(+) Anti-CCP

Dx/Tx (1st/2nd line)?

Answer 6

Rheumatoid Arthritis
Methotrexate
TNF Inhibitors (if failed MTX)
*infliximab, etanercept, adalimumab

(negative Anti- RF/CCP/ANA do not r/o RA)

Question 7

20-30s yo
Bilateral wrist/hand joint pain/swelling
Oral Ulcers

Dx/Tx?

Answer 7

SLE
Hydroxychloroquine

Question 8

50+ yo Female
Bilateral pain & morning stiffness of, Neck, Shoulders, Hips, etc.
Joints have ↓ ROM
Fatigue, weight loss, ↑ ESR
Dx/Tx?

Answer 8

Polymyalgia rheumatica
Low Dose Prednisone (Glucocorticoid)

Question 9

Methotrexate side effects (2)
screening (2)

Answer 9

Hepatotoxic
Pulmonary Fibrosis

(get initial LFTs & CXR)

Question 10

Hydroxycholoquine side effect?
Screening?

Answer 10

Retinopathy

(get yearly eye exams)

Question 11

TNF inhibitor side effects (2)

Answer 11

Malignancy (lymphoma)
Infections
(Granulomatous illness → Listeria; Legionella)

FYI: legionella tx FQ or Macrolide

Question 12

25M with low back pain that is relieved with morning exercise.
Dx/Dxt/Tx

prognosis?

Answer 12

Ankylosing Spondylitis
X-Ray of Sacroiliac joints (Bamboo/fusion)
→ If negative, MRI of Sacroiliac Joints
NSAIDs

(Normal Life Expectancy)

Question 13

29M with weeks of R knee pain, pain at the achilles tendon insertion (Enthacitis), psoriasiform lesions on the palms/soles
(+) recent h/o either:
- STI
- diarrhea
- glans penis ulcer

Dx/Tx?

Answer 13

Reactive Arthritis
NSAIDs

(only give ABxs if signs of infection: Fever, ↑ wbc, discharge)

Question 14

Acute worsening of SLE symptoms
Dx/Tx?

Answer 14

Lupus Flair
IV Corticosteroids

Question 15

Pt presents with new joint pain, pleuritic chest pain, and fever.
Recently placed on Hydralazine (Isoniazid)
Dx/Tx?

Answer 15

Drug-Induced Lupus
Stop the offending medication

(anti-Histone antibodies)
OTHERS: sulfonamides, Etanercept, phenytoin, procainamide

Question 16

Neonate presents with Bradycardia (<60 bpm)
Dx/Etiology?

Answer 16

3rd degree heart block
Maternal SLE

(anti- Rho & anti-La IgG antibodies damage fetal conducting system)

Question 17

Butterfly rash beneath the nose/lower lip that worsens with sun exposure + chronic joint pain.
Dx?

Answer 17

SLE

Question 18

SLE pt with Hgb 8 & (+) Coombs test
Dx?
T__HSR

Answer 18

Auto-immune Hemolytic Anemia

(Type 2 HSR s/t ABs against own blood)

Question 19

What SLE Antibody is associated with kidney damage/disease activity?

Answer 19

Anti DS–DNA antibodies

Question 20

30s yo Female with:
Hypercoagulable (thromboembolic) state/sequelae
or
Unexplained Fetal loss
Positive ANA ± RPR
Dx?

Answer 20

Antiphospholipid antibody syndrome

(can also present w/ Mitral insufficiency: Holosystolic apex)

RPR is a false (+)

Question 21

33F with a 2 week history of painful/burning neuropathy
(+) abdominal pain
(+) weight loss
Angiography → segmental aneurysmal dilation of the SMA & IMA.

Dx/NBSIM + classic finding?
organ spared?

Answer 21

Polyarteritis nodosa
Biopsy affected organ
Bx finding → HepB surface Ag
Lungs spared
─
(Extra info)
Tx: Steroids or HBV therapy , if (+)

Question 22

Dry eyes/mouth
Parotid enlargement
Raynauds/ Interstitial lung dz
h/o auto-immune disease
Dx/Dxt/Tx? (lower yield)

Answer 22

(dry eyes + parotids = END of STORY)
Sjögren syndrome
Pilocarpine (+ Vit D)
Artificial tears/saliva
Anti Ro/La → Salivary gland biopsy

(Autoimmune destruction of exocrine glands)

Question 23

Antihistone antibodies are specific for ____.

Answer 23

drug-induced lupus

Question 24

African American patients are most commonly affected by limited cutaneous (CREST) Systemic Sclerosis.
_____ is present in approximately 90% of patients and typically precedes pulmonary HTN sxs by several years.

Answer 24

Raynaud phenomenon

Pulmonary arterial HTN → nl PFTs

Question 25

Considerable overlap between **diffuse cutaneous SS (dcSS)** and **limited cutaneous SS (CREAST)**. Both may present with: - Raynaud phenomenon - esophageal dysmotility (+ GERD) But **dcSS** has skin thickening of the ___ & **interstitial lung disease**.

Answer 25

thickening **trunk**

Question 26

≥40F proximal muscle weakness otherwise normal neurologic exam elevated **creatine kinase** ± elevated ESR Dx/**DxT**/Tx? dx can be s/t ___ do age appropriate screening

Answer 26

Polymyositis **Muscle Biopsy** Prednisone (can be s/t paraneoplastic syndrome; do age-appropriate **cancer** screening)

Question 27

50+ y.o. Female Low grade fever + malaise Stiffness in **shoulders, hips**, neck, or extremities Elevated ESR/CRP Normal CK Dx/Tx/Association

Answer 27

Polymyalgia Rheumatica Low dose Prednisone Association w/ **giant cell (temporal) arteritis**

Question 28

Presents with **fever** and the following **triad** of symptoms: Migratory polyarthritis Erythema nodosum Bilateral hilar lymphadenopathy (fullness) Dx/**Tx**

Answer 28

Lofgren syndrome (Sarcoidosis) **Glucocorticoids**

Question 29

Review Sarcoid Features Flip card.

Answer 29

Question 30

_____ antibodies are present in **myasthenia gravis**, which is characterized by muscle fatigability (**weakness increases with repeated activity**) and involves **oculobulbar muscles** in addition to **proximal** limb muscles.

Answer 30

Acetylcholine receptor

Question 31

LOW YIELD: syndrome characterized by **recurrent oral and genital ulcers** + Skin and ocular problems MCCOD: **Thrombosis**

Answer 31

Behçet syndrome

Question 32

Long-term cyclophosphamide use is associated with what 2 negative side effects?

Answer 32

**Hemorrhagic cystitis** bladder cancer

Question 33

Myopathies * Dermatomyositis * Polymyositis * Inclusion body myositis present with muscle weakness w/o pain ↑ CK EMG: **polyphasic, short-duration, low-amplitude APs** First-line tx:

Answer 33

Glucocorticoids

Question 34

Symmetric **proximal** muscle weakness + **skin** findings * Upper eyelids (heliotrope rash) * Extensor surfaces of the hands rash ↑ CK EMG: **polyphasic, short-duration, low-amplitude APs**

Answer 34

Dermatomyositis (F>M)

Question 35

50 yo Male pt Asymmetric **proximal & distal** muscles weakness ↑ CK EMG: **polyphasic, short-duration, low-amplitude APs**

Answer 35

Inclusion body myositis (M>F)

Question 36

Symmetric **proximal** muscle weakness **w/o** skin findings ↑ CK EMG: **polyphasic, short-duration, low-amplitude APs**

Answer 36

Polymyositis (F>M)

Question 37

For TNF inhibitors (RA or IBD pt on Infliximab, etc.) Bugs to screen for before starting: Malignant complication with chronic use: 2 classic opportunistic infections: Tx for RA when pregnant:

Answer 37

Screen for Hep B, TB, Hep C, HIV Chronic use → Lymphoma risk Opportunistic infx → Legionella or Listeria (Granulomatous illness) RA pregnancy tx→ Hydroxychloroquine

Question 38

LUQ fullness and WBC of 1k in a pt with Rheumatoid Arthritis dx?

Answer 38

Felty Syndrome (Triad: RA + Splenomegaly + neutropenia)