Rheum Pt Three- Paulson Flashcards
Juvenile Idiopathic Arthritis (JIA) is basically RA in _________
kids
JIA is a spectrum of chronic arthritides in patients who are _______ years old that involve at least _______ joint and have lasted for at least ______ weeks
≤ 16, 1 joint, 6 weeks
How many joints are involved in Oligoarticular JIA aka pauciarticular JRA?
less than or equal to 4 joints
What joint is mc involved in Oligoarticular JIA aka pauciarticular JRA?
Knees
What is a lab that will be elevtaed in Oligoarticular JIA aka pauciarticular JRA?
+ ANA (60%) and ESR/CRP may be mildly elevated
Those kiddos with Oligoarticular JIA aka pauciarticular JRA are at inscreased risk of ________ and must have regular _____-______ exams until the age of 18
uveitis, slit-lamp exams
Oligoarticular JIA aka pauciarticular JRA is often symmertric or asymmetric joint invovlement?
Asymmetric
A patient and child comes in and the mother states she has noticed that the child is all of a sudden walking funny and she has swelling in the knee. What should you be suspicious of?
Oligoarticular JIA aka pauciarticular JRA
Polyarticular JIA involves how many joints?
greater than or equal to 5
What lab is typically elevated in Polyarticular JIA?
ESR usually pretty high and pt will have a positive RF
Seropositive polyarticular JIA resembles the adult version of what disease?
RA and positive RF
Seropositive polyarticular JIA is symmertric or asymmeteric?
Symmetric arthritis, usually the small joints of hands and feet
Does seropositive polyarticular JIA have extraarticular invovlement?
Yes: rheumatoid nodules, Felty syndrome, vasculitis, lung disease can occur.
This type of polyarticular JIA is common in younger childre, affects larger joints (knees, ankles, wrists) and has NO extraarticular features
Seronegative polyarticular JIA
A patient presents with a fever that seems to wax and wane throughout the day and the patient has developed a “salmon-colored evanescent macular rash.” What disease should you be concerned about?
Systemic Onset JIA (SOJIA) aka Still’s disease
Paitents with Systemic Onset JIA (SOJIA) aka Still’s disease can have _______ which can be fatal
MAS (macrophage activation syndrome)
This type of arthritis involves at least 1 joint (often in the small or medium joints and is asymmetric). It can be common to see these as well: • DIP synovitis • Dactylitis • Nailpitting • Psoriatic rash or FH of psoriasis
Psoriatic arthritis
Psoriatic arthritis can also be associated with acute anterior ________ so patients need regular screenings
uveitis
This type of jeuvenile arthritis causes sacroiliac tenderness and is associated with HLA-B27. You will commonly see inflammation at site of attachment of tendon/ligament to bone
Enthesitis-related JIA
Are labs helpful in the dx of juvenile arthritis?
—Clinically diagnosed
• No specific lab or imaging test to definitively confirm diagnosis, though they can guide you in the right direction.
• Exclude other possibilities
What are initial mainstays of therapy for juvenile arthritis while the patient is being referred to rheum?
NSAIDs & intraarticular joint injections
(Ibuprofen, Naproxen)
-intraartic triamicolone acetate
Patients with SOJIA may need this type of treatment for initial symptom control?
Steroid
What is a last resort tx for jeuvenile arthritis?
- DMARD (Methotrexate)
- Biologics (Infliximab)
Patients with MAS would need what treatment urgently?
Cephalosporins & steroids
Which juvenile arthritis has the highest mortality?
SOJIA have the highest mortality
This is a chronic, systemic autoimmune disorder with fibrosis of the skin and other
organs and it literally means “hard skin”
Systemic Sclerosis (Scleroderma)
What is CREST syndrome? What does it stand for?
It is the limited (better outcome) form of Scleroderma
C- Calcinosis cutis R-Raynauds E- Esophogeal immotility S- Sclerodactyly T- Telangictasias
What type of scleroderma has a worse outcome?
Diffuse type
Raynauds is often the first sign of what disease?
Scleroderma
When you see “watermelon stomach” think of what disease?
Scleroderma
Pericarditis is a common manifestation in what disease?
Scleroderma
This clinical manifestation of Scleroderma can lead to malignant HTN and can lead to death
Scleroderma renal crisis (SRC)
What labs are elevated in scleroderma?
- ANA usually positive (>95%)
- Anti-SCL-70 positive in 1/3 with diffuse disease and 20% with CREST
- Poor prognosis
What imaging should be done in a patient with scleroderma to detect lung changes? What other test should be done to check lungs?
CT, PFTs
What imaging should be done to monitor a patient with scleroderma for pHTN?
echo
Treatment for scleroderma?
Treatment is targeted to organs affected
Treatment for scleroderma in a patient with diffuse skin involvement and/or systemic involvement?
Immune process targeted: glucocorticoids, methotrexate, mycophenolate mofetil (MMF), cyclophosphamide, IVIG
Treatment for scleroderma in a patient with Raynauds?
CCB (nifedipine)
______ disease is the leading cause of mortality in scleroderma?
lung
This autoimmune disease is most commonly known for fatigue, joint pain and a rash
SLE (Lupus)
Lupus has the highest prevalence in _________ females
childbearing
Butterfly rash sparing the nasolabial folds and triggered by the sun?
Acute cutaneous lupus erythematosus (ACLE)
Which type of lupus is the most photosensitive of all SLE rashes?
Subacute cutaneous lupus erythematosus (SCLE)
AKA discoid lupus with raised, erythematous plaques with a scale and commonly with mucosal ulcerations
Chronic cutaneous lupus erythematosus (CCLE)
This organ is commonly involved in lupus?
Renal –lupus glomerulonephritis
What is the mc eye manifestation in lupus?
Keratoconjunctivitis sicca
What labs would you expect to see in lupus?
- ANA almost always positive
- Anti- dsDNA
- Anti-Sm
What are some medications that can cause drug-induced lupus?
Procainamide, hydralazine, isoniazid, chlorpromazine, methyldopa, minocycline, quinidine
“Please hide in car my mini quinidine” mnemonic
Lab abnormalities and clinical manifestations usually normalize when drug is discontinued
Treatment for patient with cutaneous lupus?
Initial: topical glucocorticoids. Start low potency >medium -> high
May need hydroxychloroquine if need systemic meds
Treatment for patient with musculoskeletal lupus?
NSAIDs & Tylenol
May add hydroxychloriquine
Treatment for patient with serositis (lung/cardiac?) lupus?
NSAIDs and/or low-mod glucocorticoids
May add hydroxychloriquine or PO colchicine if persistent or recurrent
Autoimmune myopathy characterized by proximal muscle weakness
Polymyositis
How do you differentiate between PMR and Polymyositis?
PMR mostly associated with pain and polymyositis is generally just muscle weakness
This disease causes a symmetric, gradual and progressive leg weakness that spreads to the arm with proximal muscle weakness and fatigue.
Polymyositis
CK will be elevated in which disease?
Polymyositis
What is the gold standard test for Polymyositis?
Muscle biopsy is the gold standard for inflammatory myopathies
What is the treatment for Polymyositis?
Prednisone with osteoporosis prophylaxis
This type of arthritis is associated with psoriasis
Psoriatic arthritis
You may see nail pitting, ridging, and onycholysis in this type of arthritis?
Psoriatic arthritis
“pencil cup deformity” is common in which disease?
Psoriatic arthritis and imaging (xray) would show this
What term is this and what disease is it associated with?
Deforming, destructive arthritis
Arthritis mutilans
Psoriatic arthritis
What term is this and what disease is it associated with?
Sacroiliitis & spondylitis
Spondyloarthritis
Psoriatic arthritis
What term is this and what disease is it associated with?
Inflammation at the site of insertion of tendons
Enthesitis
Psoriatic arthritis
What term is this and what disease is it associated with?
Flexor tendons of hands, extensor carpi ulnaris, & others
Tenosynovitis
Psoriatic arthritis
What term is this and what disease is it associated with?
“Sausage digit”- diffuse swelling of an entire finger or toe
Dactylitis
Psoriatic arthritis
What imaging do you get for psoriatic arthritis?
xray
Medication for a patient with psoriatic arthritis with less than 4 joints involved and patient isnt in much discomfort?
NSAIDs (Naproxen, Celebrex)
Medication for a patient with psoriatic arthritis and more severe and unresponsive to NSAIDs
DMARDs (Methotrexate or sulfasalazine), Biologic TNF inhibitors (etanercept, infliximab)
Beware of treating psoriatic arthritis and immunosuppression because why??
Immunosuppression, reactivation of latent TB
What medication should NOT be used in a psoriatic arthritis patient?
Don’t use PO steroids- can cause a flare of pustular psoriasis