Rheum Part 3-Paulson (Exam 2)

Question 1

A child might have a form of JIA if they have at least ___ affected joint(s) for ___ week(s).

Answer 1

at least 1 joint

for at least 6 weeks

Question 2

JIA can manifest in many different ways, but the main mechanism of the disease if ________________________.

Answer 2

persistent joint swelling that comes from synovial fluid increase (with inflammatory cells) + expansion of pannus=joint damage

Question 3

This subtype of JIA is most common in ages 1-7yrs, involves 4 joints or less (asymmetrically); most commonly seen in the knee joint. This kiddos might also have prominent morning stiffness.

Answer 3

Oligoarticular JIA (aka pauriarticular JIA)

Question 4

Children with oligoarticular JIA (esp ones with positive ANA) have an increased risk of developing what type of eye disorder?

Answer 4

Uveitis (can be asymptomatic and/or occur without the joint symptoms)

Question 5

This subtype of JIA affects 5 or more joints and typically has an elevated ESR.

Answer 5

Polyarticular JIA

Question 6

If a child with polyarticular JIA has a positive Rf, it is referred to as ____________.

Answer 6

Seropositive

Question 7

___________(seropositive or seronegative) is the more aggressive form of polyarticular JIA and resembles the adult version of RA.

Answer 7

Seropositive

Question 8

T/F? Pt’s with Seronegative polyarticular JIA usually have other extraarticular sx’s like rheumatoid nodules, vasculitis, and lung dz.

Answer 8

False. Seronegative does not have extraarticular sx’s, while seropositive does.

Question 9

The other/ technical name for “Still’s Disease is _________________.

Answer 9

Systemic Onset JIA (SOJIA)

Question 10

Children with SOJIA are likely to have a daily ________ fever. What does this mean?

Answer 10

Daily quotidian fever, is a fever that spikes then resolves spontaneously, can go up and down

Question 11

If you see a child with a “salmon-colored evanescent macular rash” on their trunk and extremities, what rheumatologic disease would you think about?

Answer 11

Systemic Onset JIA (SOJIA)

Question 12

You are working up an ill-appearing 10 yr old child, who you suspect has SOJIA due to his clinical presentation. You decide to run labs. What labs do you choose and what do you expect them to reflect if he does indeed have SOJIA?

Answer 12

Labs: CBC, ESR and Iron panel

Results would show: High leukocytosis, very high ESR, elevated ferritin

Question 13

______ ________ syndrome is a potentially fatal complication that can occur in kiddos with SOJIA. This syndrome occurs due to excess circulating ___________, which can cause seizures/DIC/bleeding.

Answer 13

MAS (Macrophage activation syndrome)

Cytokines

Question 14

The following are characteristics of what type of JIA? Arthritis in at least 1 joint, DIP synovitis, dactylitis, nail pitting.

Answer 14

Psoriatic Arthritis

Question 15

With enthesitis-related JIA, you would expect tenderness of the ____________ and a positive HLA-B27.

Answer 15

Sacroiliac

Question 16

Which type of JIA includes those with juvenile-onset spondylitis, reactive arthritis, and arthritis associated with IBD?

Answer 16

enthesitis-related JIA

Question 17

How do you diagnose JIA?

Answer 17

Clinically diagnosed; no specific lab/imaging to definitely confirm the diagnosis’, but they can guide you in the right direction and exclude other possibilities.

Question 18

What are the mainstays of treatment for JIA?

Answer 18

NSAIDS (ibuprofen, naproxen, meloxicam)
Joint injections (triamcinolone acetate)

Question 19

Children with SOJIA may need a __________ intially for sx control.

Answer 19

steroid

Question 20

Pt’s with which type of JIA should be treated aggressively with a DMARD?

Answer 20

Seropositive polyarticular JIA

Question 21

Which form of JIA has the highest mortalilty?

Answer 21

SOJIA

Question 22

_______ ________ is a chronic, systemic autoimmune disorder with fibrosis of the skin and other organs.

Answer 22

Systemic sclerosis (Scleroderma)

Question 23

There are 2 forms of Scleroderma; _________ which includes CREST syndrome, and _________.
Which is more common?

Answer 23

Limited (affects face, distal to knees/elbows)

Diffuse (throughout he body)

MC=Limited (80%)

Question 24

What does CREST Syndrome consist of?

Answer 24

Calcinosis cutis- Ca deposits in the skin
Raynaud phenomenon(usu 1st sign, can start years before any other sx’s)
Esophageal dysmotility
Sclerodactyly-thickening/tightening of skin on toes/finger
Telangiectasia

Question 25

There is a higher incidence of scleroderma in which two specific populations?

Answer 25

African americans | Choctaw Native Americans

Question 26

What are some common skin manifestations of scleroderma?

Answer 26

``` Initially, edema + pruritis Later, thick, hide-like skin Hyperpigmentation or depigmentation Digital ulcers Pitting of fingertips Loss of appendicular hair Telangiectasias Calcinosis ```

Question 27

Scleroderma can also manifest in other important organs. One thing that has been found on autopsy is vascular ectasia of the stomach antrum, aka ___________ ___________.

Answer 27

Watermelon stomach

Question 28

________ _______ ______ is a complication of Scleroderma and is the sudden onset of malignant HTN that can lead to renal failure and death.

Answer 28

Scleroderma Renal Crisis (SRC)

Question 29

With Scleroderma pt's will have a ________(pos/neg) ANA 95% of the time. _________ is another lab that is positive in 1/3 of pt's with diffuse dz and 20% with CREST (Indicates poor prognosis).

Answer 29

POSITIVE Anti-SCL-70

Question 30

What is the tx for scleroderma?

Answer 30

No proven disease-modifying drug to treat scleroderma. | Treatment is targeted to organs affected.

Question 31

How would you treat a scleroderma pt with diffuse skin involvement and/or systemic involvement?

Answer 31

Immune process targeted: glucocorticoids, methotrexate, mycophenolate mofetil (MMF), cyclophosphamide, IVIG

Question 32

What class of meds is used to treat Raynaud Phenomenon?

Answer 32

Calcium Channel blockers (Long-acting nifedipine 30-180 mg daily or Sildenafil 50 mg PO BID)

Question 33

What is the leading cause of mortality in pts with Scleroderma?

Answer 33

Lung disease | CHF and CKD also common

Question 34

____________ ______ __________ is a Chronic inflammatory disease with autoantibodies and varying presentations that can affect every organ.

Answer 34

Systemic Lupus Erythematosus (SLE)

Question 35

SLE is more prevalent in which population?

Answer 35

Childbearing females

Question 36

SLE can have many different manifestations. If a pt presents with a localized malar "butterfly" rash that spares the nasolabial folds (often triggered by sun exposure) OR generalized maculopapular lesions in a photosensitive distribution of any part of the body, they would be said to have ______ ________ ______ _________.

Answer 36

Acute cutaneous lupus erythematosus (ACLE)

Question 37

________ _________ _________ __________ is the most photosensitive of all SLE rashes and includes scaly, erythematous papules of the torso/limbs, but spares the face.

Answer 37

Subacute cutaneous lupus erythematosus (SCLE)

Question 38

_______ ________ _________ _________ presents with discoid lupus: raised erythematous plaques with a scale/raised erythematous ring around a lesion.

Answer 38

Chronic cutaneous lupus erythematosus (CCLE)

Question 39

________ lupus is the most common subtype of CCLE, and consists of raised, erythematous plaques with a scale. Raised erythematous ring around the lesion. Lead to scarring, skin atrophy, as well as Lupus panniculitis.

Answer 39

Discoid

Question 40

In SLE, pt's can have MSK sx's like __________ and __________ arthritis.

Answer 40

Arthralgia Nonerosive (arthritis)

Question 41

SLE commonly involves the renal system; there are several types of lupus ______________.

Answer 41

glomerulonephritis | **Renal involvement is a poor prognostic factor.

Question 42

SLE pt's can also have involvement of the GI system, lungs and heart. What are the most common presentation of each of these symptoms?

Answer 42

GI- dysphagia lungs- pleuritis cardiac- pericarditis

Question 43

Pts with SLE are at risk for an atypical cardiac condition called _________ _________ ___________, which can lead to arrhythmias.

Answer 43

Atypical verrucous endocarditis (Libman-Sacks)

Question 44

What are the most common presentations of the eye, lymph and blood in pt's with SLE?

Answer 44

Eye- keratoconjunctivitis (secondary Sjorgens syndrome possible) Lymph- LAD; commonly cervical, axillary, and inguinal nodes. Blood- Anemia of chronic dz is most common. Leukopenia and mild thrombocytopenia is also common.

Question 45

Name 4 antibody labs that can be ordered in pt's you suspect to have SLE. What is the specificity/ purpose of each?

Answer 45

1. ANA almost always positive, but not specific for SLE. 2. Anti-dsDNA- Specific but not sensitive. Levels often correspond to disease activity. 3. Anti-Sm (anti-Smith)- 99% specific, but not very sensitive. 4. Antiphospholipid antibodies- Lupus anticoagulant. Can prolong aPTT. **False positive for syphilis

Question 46

Name some drugs that can cause Drug-induced lupus?

Answer 46

Procainamide, hydralazine, isoniazid, chlorpromazine, methyldopa, minocycline, quinidine

Question 47

Ways to differ b/w SLE and drug-induced lupus? (3 ways)

Answer 47

If drug-induced: 1. Doesn’t normally have nephritis and CNS involvement 2. No anti-dsDNA antibodies or hypocomplementemia 3. Lab abnormalities and clinical manifestations usually normalize when drug is discontinued

Question 48

How do you diagnose SLE?

Answer 48

``` ACR criteria (4 out of 11) or SLICC criteria (4 out of 17) or if pt doesnt meet either of these criteria, you can still dx if pt has 2-3 criteria plus at least 1 other feature assicated with SLE: Optic neuritis, Aseptic meningitis, Pneumonitis, pulmonary hemorrhage, pHTN, ILD, Myocarditis, Libman-Sacks endocarditis, Abdominal vasculitis, Raynaud phenomenon, Elevated ESR/CRP ```

Question 49

ACR Criteria for SLE

Answer 49

4 out of 11 - malar rash - photosensitivity-skin rash - discoid rash - oral ulcers-usu painless - arthritis-nonerosive,2+ peripheral joints - serositis-pleuritis/pericarditis - renal disorder - neuro disorder-sz/ psychosis - hematologic disorder - ANA - immunologic disorders- anti-DNA or anti-Sm OR antiphospholipid antibody

Question 50

SLICC Classification Criteria

Answer 50

``` 4 out of 17 Acute cutaneous lupus Chronic cutaneous lupus Nonscarring alopecia Oral or nasal ulcers Joint disease Serositis Renal Neurologic Hemolytic anemia Leukopenia or lymphopenia Thrombocytopenia ANA Anti-dsDNA Anti-Sm Antiphospholipid Low complement Direct Coomb’s test ```

Question 51

What are some general tx considerations for SLE?

Answer 51

- exercise - smoking cessation - sun protection (DAILY sunscreen of 55+ SPF) - healthy diet - prevent glucocorticoid-induced osteoporosis--bisphosphanates? - many will need to be treated w antimalarials (hydroxychloroquine)

Question 52

How would you treat cutaneous lupus sx's? MSK sx's? Serositis sx's?

Answer 52

Cutaneous- intially topical glucocorticoids (start low then move up). Can do intralesional injections for triamcinolone for refractory lesions. Some may need systemic meds (hydroxychloroquine) MSK--NSAID or Tylenol are 1st line. May add hydroxychloroquine. Serositis-- NSAID and/or low-mod glucocorticoids. Hydroxychloroquine or PO colchicine if persistent/recurrent

Question 53

How would you treat a pt with SLE who has thrombocytopenia <20k or <50k with hx of bleeding and severe autoimmune hemolytic anemia?

Answer 53

- high dose glucocorticoids, then taper once improvement seen. - IVIG often used - immunosuppressants - splenectomy (last resort)

Question 54

What are the commonly used immunosuppressants for SLE?

Answer 54

``` Glucocorticoids hydroxychloroquine methotrexate azathioprine mycophenolate mofetil cyclophosphamide ```

Question 55

T/F? Pt's with SLE have a 5x higher incidence of MI than general population.

Answer 55

True, due to Atherosclerosis from chronic inflammation

Question 56

__________ is a rare autoimmune myopathy characterized by proximal muscle weakness

Answer 56

polymyositis

Question 57

What symptoms might a pt with polymyositis present with?

Answer 57

Proximal muscle weakness and muscle fatigue (not usu painful) - usu gradual and progressive - symmetric - may c/o trouble rising from chair, climbing stairs, washing hair

Question 58

In a pt with polymyositis, what particular lab would you expect to be elevated? Which lab is most helpful for detecting cardiac involvement? What other labs might also be elevated?

Answer 58

CK Troponin LDH, aldolase, AST, ALT

Question 59

What is the gold standard for diagnosing inflammatory myopathies?

Answer 59

``` muscle biopsy (of muscle thats weak/affected, but not atrophied--quads or deltoids are good choices) will show inflammatory infiltrates invading fascicles ```

Question 60

What could an EMG and MRI help you with/show you when working up a pt with possible polymyositis?

Answer 60

Good to identify which muscle you want to biopsy - Do bx on contralateral side to avoid needle artifact - MRI might be even better than EMG at this - Helps differentiate myositis from a neuropathic cause for weakness - EMG typically shows early recruitment - Increased number of motor units firing rapidly to produce a low level of contraction - MRI can show areas of muscle inflammation, edema, fibrosis, calcification

Question 61

How do you make a diagnosis or polymyositis?

Answer 61

No validated diagnosis/classification criteria. Can make the diagnosis without a muscle biopsy if the patient has: Characteristic clinical symptoms Proximal muscle weakness Abnormal muscle enzyme labs No better explanation for a diagnosis

Question 62

What is the treatment for polymyositis?

Answer 62

- Initially steroids-Prednisone 1 mg/kg/day PO daily (max 80 mg) 4-6 weeks, taper slowly based on tx response/labs. - osteoporosis prophylaxis Frequently combine w/another immunosuppressant, either initially or later if no response to steroids. Azathioprine (2 mg/kg/day) or Methotrexate (up to 25mg/day). PT recommended.

Question 63

T/F? Most pt's with polymyositis don’t regain completely normal muscle function.

Answer 63

true also, worst prognosis in those who had delayed tx. *Fun fact- Those with anti-Jo-1 antibodies usually don’t completely respond to treatment & do worse in general.

Question 64

In adult pt's with _________ arthritis, it can be difficult to distinguish from RA.

Answer 64

Psoriatic

Question 65

Which presentation of psoriatic arthritis is more common, symmetric polyarthritis or asymmetric oligoarthritis?

Answer 65

symmetric polyarthritis

Question 66

An adult pt presents with DIP joint pain/swelling and cervical spine pain, as well as morning stiffness greater than 30 mins. What type of arthritis comes to mind?

Answer 66

Psoriatic

Question 67

Pts with psoriatic arthritis can have ______ _______, which is a deforming, destructive arthritis.

Answer 67

Arthritis mutilans

Question 68

Psoriatic arthritis can also involve the spine, referred to as ___________.

Answer 68

Spondylarthritis

Question 69

Psoriatic arthritis can also cause inflammation at the sits of insertion of tendons causing soft tissue swelling. What is this condition referred to as?

Answer 69

enthesitis

Question 70

________ or inflammation of the flexor tendons of hands, as well as __________ or diffuse swelling of an entire finger or toe, are also signs of psoriatic arthritis.

Answer 70

Tenosynovitis Dactylitis "sausage digit"

Question 71

What signs might you see on a pt's nails if they have psoriatic arthritis?

Answer 71

Pitting Ridging Onycholysis Nail bed hyperkeratosis Splinter hemorrhage

Question 72

The "pencil-in-cup" appearance on xray, is indicative of what type of arthritis?

Answer 72

Psoriatic

Question 73

How do you make a diagnosis of psoriatic arthritis?

Answer 73

``` No consensus on diagnostic criteria. Generally, diagnosis made in a patient with both psoriasis & inflammatory arthritis in a typical pattern. Can diagnose it in the absence of psoriasis if: Distal joint involvement Asymmetric distribution Nail lesions Dactylitis FH of psoriasis Presence of HLA-C*06 ```

Question 74

What is CASPAR criteria?

Answer 74

Used to dx patient with an inflammatory MSK disease with PsA if they have at least 3 points from the following: -Skin psoriasis that is: Present (2 points) Previously present by hx (1 point) FH of psoriasis, if pt not affected (1 point) -Nail lesions (1 point) -Dactylitis (present or past) (1 point) -Negative RF (1 point) -Juxtaarticular bone formation on radiographs (1 point)

Question 75

When treating psoriatic arthritis, what medication do you want to avoid? Why?

Answer 75

PO steroids | Can cause flare of pustular psoriasis

Question 76

How do you treat psoriatic arthritis?

Answer 76

``` -Mild Peripheral or axial arthritis (<4 joints/ no damage seen on xrays) NSAIDS 1st choice Naproxen, Celebrex -More severe or unresponsive to NSAIDS: DMARDs Methotrexate, sulfasalazine, azathioprine, cyclosporine, hydroxychloroquine -Biologic TNF inhibitors Etanercept, infliximab, adalimumab, golimumab, certolizumab pegol ```