RHEUMATOLOGY Flashcards
A patient has high spiking fevers daily for the last 2 weeks. What is your differential diagnosis? (5)
-workup?
- Infection
- Kawasaki disease
- Periodic fever syndromes
- Malignancy (especially leukemia and lymphoma)
- Systemic JIA
Work-up:
- Full infectious workup
- Bone marrow aspirate PRIOR to starting corticosteroid treatment for query JIA
A patient presents to you with dactylitis. What is your differential diagnosis? (3)
- Sickle cell anemia
- JIA (ie. enthesitis related, psoriatic arthritis)
- Trauma
A patient presents to you with isolated Raynaud phenomenon. What are the 2 best predictive factors for future development of autoimmune diseases?
- Positive ANA
- Abnormal nail fold vasculature
A patient presents to you with leg pain that occurs solely at night. What is your differential diagnosis? (3)
- Malignancy: osteosarcoma, Ewing sarcoma
- Osteoid osteoma (benign)
- Growing pains
Antibiotic choice for osteomyelitis/septic arthritis:
- neonate
- 1-3 month old
- child
- teenager -sickle cell
-Neonate: Cloxacillin and Gentamicin
-1-3 month old: Cefuroxime (because of more gram negatives)
- Child: Cefazolin
- Teenager: ceftriaxone OR cefixime + azithromycin
- Sickle cell: cefotaxime (salmonella coverage)
What is the diagnostic criteria for Behcet disease?
Needs:
- Recurrent oral ulcers AND 2 or more of the following:
a. recurrent genital ulcers
b. eye lesions (uveitis)
c. skin lesions (erythema nodosum, pseudo vasculitis, etc.)
d. Pathergy (skin papule 2 mm or more in size developing 24-48 hrs after insertion of 20-25 gauge needle into the skin)
Differential diagnosis for fever and limb pain?
Reactive: -arthritis -transient synovitis Infections: -Lyme disease -Viral Malignancy Inflammatory
Features of transient synovitis vs. septic arthritis?
Septic arthritis:
Clinical;
lots of pain, fever,
MARKED RESISTANCE TO MOBILITY AND SWELLING/WARMTH OF JOINT
Labs: elevated ESR/CRP, elevated WBC,
Aspiration: positive gram stain or culture of joint fluid, key is
Transient synovitis:
Clinical will still be active but limping, looks well, can elicit range of motion in joint if done gently, can have low fever or afebrile,
Labs normal WBC, mild pain usually, normal or mildly elevated ESR/CRP
****Remember that transient synovitis is a diagnosis of exclusion
How does colchicine work in FMF?
Binds to microtubules to prevent activation, proliferation and functioning of inflammatory cells
What are the clinical features of familial mediterranean fever? -hallmark?
-treatment?
- Fever episodes x 1-3 days q4-8 wks (very predictable)
-
Clinical:
a. Serositis - CLINICAL HALLMARK = peritonitis, pleuritis, synovitis
b. Skin: erysipelas-like rash on shins and dorsum of feet
c. MSK: monoarthrtis, myalgia
N.B. cause of AA-Amyloidosis without predisposing disease,
Treatment: colchicine
- Diagnosis: typically clinical (recurrent short fevers in right demographic, accompanied by abdominal/chest/skin/joint manifestations.
- Tel HashomerCriteria: diagnosis with > 2 major criteria, or 1 major and > 2 minor:
- Major: recurrent fever with serositis, AA-Amyloidosis without predisposing disease, favorable response to colchicine treatment.
- Minor: recurrent febrile episodes, erysipelas-like erythema, FMF in a first-degree relative.
FMF What is the epidemiology of FMF?
Genetics, race, age
Most common monogenic fever syndrome
Autosomal recessive mutations in the MEFV gene on chromosome 16p
Most common in Ashkenazi Jewish, Arab, Turkish, and Italian populations.
Onset of disease typically in adolescence or childhood (> 90% start before 20y)
How long to treat with IV antibiotics in osteomyelitis/septic arthritis?
1. iv Abx: Until fever resolves > 24 hrs, pain completely resolved (may need up to 4-6 weeks);
- then switch to PO abx x 2-3 wks
3. Monitor CRP and ESR for improvement
What does the prognosis of HSP depend on?
Depends on severity of nephritis (worse prognosis with nephrotic syndrome)
-end stage renal disease occurs in 1-3% of patients
What is the diagnostic criteria for HSP?
Need: Palpable purpura or petechiae with lower limb predominance AND 1 or more of the following:
- Diffuse colicky abdominal pain with acute onset (can include intussusception, GI bleed) = 50-75% of patients
- Skin biopsy showing IgA deposits in leukocytoclastic vasculitis OR kidney biopsy showing proliferative glomerulonephritis with IgA deposits (THIS IS REQUIRED IF PURPURA IS IN ATYPICAL DISTRIBUTION)
- Arthritis- oligoarticular or arthralgia of acute onset (75% of patients usually knees/ankles with edema)
- Renal involvement: proteinuria, hematuria impaired renal function
- CNS: headache, fits or ataxia
What is the most common vasculitis in children?
HSP
What are the diagnostic criteria for juvenile dermatomyositis? (5)
- what can be seen on capillaroscopy?
- what organ systems aside from MSK/neuro are involved?
-
Myositis: (you need 2 of the following)
a. Symmetrical proximal muscle weakness
b. Elevated muscle enzymes (CK, AST, LDH, aldolase)
c. Abnormal EMG demonstrating denervation and myopathy
d. Abnormal muscle biopsy demonstrating necrosis and inflammation
e. MRI - proximal muscle inflamtion -
Characteristic skin changes: NEEDED IN CRIETERIA
a. Gottron’s papules on dorsal surface of the knuckles,
b. heliotrope rash over the eyelids
c. Shawl - V sign sun areas
d. Ulcerations at Gottrons papules/corners of eyes
e. Calcinosis in subcutaneous and deep nodules - Joint: Polyarticular arthritis/joint contractures
-
Vascular;
a. Dilated/tortuous nail fold capillaries (tortuosity, dilatation, dropout)- capillaroscopy
b. Raynauds:
5. Other organ systems:
a. Cardiac: cardiomyopathy (rare)
b. Interstitial lung disease ( poor prognostic sign)
c. GI tract symptoms: GI vasculopathy ranges on a spectrum from abdominal pain to ischemic bowel (with perforation and ulceration described).
d. Lipodystrophy: loss of subcutaneous fat, accompanied by metabolic syndrome.
JDM WHAT ARE THE INVESTIGATIONS YOU CAN ORDER?
- Muscle Enzymes:at least one is elevated in 80-98% of patients at time of diagnosis
- CK, AST, ALT, and LDH are main ones we order || Aldolase helpful but not done in most institutions
- CK typically elevated to the 1.5-15x range (contrast DMD typically much higher)
- Does not necessarily correlate with disease activity
- Can be falsely normal with progressive inactivity or severely delayed diagnosis
- vWFAntigen: marker of endothelial injury, and elevated in vasculopathy / vasculitis
•Is the best marker of disease activity and early flare
- Other Biomarkers:ESR > CRP; low complements on occasion; high IgG on occasion
-
Other Rheumatology Things:ANA, dsDNA, ENA, UA, etc…
- ANA elevated in 40-70%
- ENA positive in up to 75% (RNP, SSA, PM-Scl)
Jo-1 (2-5%): prototypicantisynthetaseantibody, associated with weakness, ILD, arthritis, and RP. High mortality.
Reference only!
Mi2 (2-13%): mild JDM with rashes and high CK; more common in Hispanics.
SRP (1%): acute and severe weakness, cardiac disease, and RP; refractory; more common in African-American girls.
Jo-1 (2-5%): prototypic antisynthetas eantibody, associated with weakness, ILD, arthritis, and RP. High mortality.
JDM What is the acute management of JDM?
- Consider admission, NPO & with IV fluids, 1:1 nursing care, and full CRM to assess cardiopulmonary weakness-may have respiratory muscle weakness; as such they may be unable to manifest tachypnea and will drop their minute ventilation rapidly. Strongly consider a baseline gas and reassess as needed to prevent need for intubation.
- Other Services: OT/PT for rehabilitation, SLP for swallowing assessment (clinical determination), and nursing for support / care.
3. Medication:
a. Corticosteroids: typically give a pulse in all but the most mild of cases, followed by a very slow taper.
b. Methotrexate: Gold standard for the management of JDM, recommended in all cases that don’t require more aggressive treatment. Minimum of 2-3 years. SQ is preferred due to concerns of absorption with subclinical GI vasculopathy.
c. IVIG: Gold standard for severe cutaneous disease; used in addition to methotrexate and steroids, not in lieu of.
d. Cyclophosphamide: used for the more severe or refractory disease manifestations, particularly those requiring ICU admission or with progression while on methotrexate.
e. Rituximab:considered at same level (or as next step, depending on algorithm) to cyclophosphamide; may be more useful in antibody-positive disease.
f. Other:Anti-TNF biologics, Imuran, MMF, and Cyclosporine are all considered for multiple refractory disease states.
What is the treatment for juvenile dermatomyositis?
-what are the 2 best predictors of longer time to remission?
- Supportive: nutrition, physiotherapy, sunscreen for photosensitive rash
-
Medications:
a. -High dose steroids 1-2 mg/kg/day with slow taper
b. -Methotrexate milder disease
c. Hydroxychloroquine for skin
d. if refractory or resistant: IVIG, cyclosporine, or rituximab
-2 predictors for longer time to remission:
- Nail fold abnormalities 2. Persistence of rash
What specific auto-antibodies (3) can often be seen with juvenile dermatomyositis?
- Anti-Jo1
- Anti-SRP
- Anti-Mi-2
What level is considered a positive ANA?
-when should specific antibodies (ie. anti-dsDNA) be ordered?
ANA >1:160 Positive & low titre
ANA >1:340 Positive & high titre
-specific antibodies should only be ordered if ANA is positive AND a disease other than JIA is suspected (ie. SLE)
A patient with JIA has a positive ANA.
What 3 things does this put them at risk for?
- Earlier disease onset
- Asymmetric arthritis (oligoarticular + psoriatic)
- Uveitis
A patient with JIA or SLE suddenly becomes extremely unwell with fever, splenomegaly, and pancytopenia. What is the most worrisome possible diagnosis?
Macrophage activation syndrome
A young female patient of yours oligoarticular JIA with positive ANA. What does she have a high risk of developing?
-what is the chance that a patient with oligoarticular JIA will have a positive ANA?
Asymptomatic uveitis -need ophtho consult
-positive ANA is found in 70% of patients with oligoarticular JIA
How useful is rheumatoid factor (RF) in diagnosis of JIA?
-implication for prognosis?
Not useful at all!
- present in 85% of adults with rheumatoid arthritis but only 5-10% of children with JIA
- helpful in classification and prognosis of JIA but should NOT be used as screening test since arthritis is a clinical diagnosis
- RF positive JIA = higher risk of aggressive joint disease with erosions and functional disability
- children with RF negative polyarthritis are typically younger and have a much better prognosis
What are 3 long term orthopedic complications of JIA?
- Leg length discrepancy
- Joint deformity
- Flexion contracture
What are the 7 types of JIA?
-definition of JIA (2 criteria)?
JIA: needs to begin before the 16th birthday and persists for > 6 weeks!!!
- Oligoarthritis 2. Polyarthritis (RF negative) 3. Polyarthritis (RF positive) 4. Enthesitis related arthritis 5. Systemic JIA 6. Psoriatic arthritis 7. Undifferentiated arthritis
JIA What are the drugs used to treat it? (5)
- Corticosteroid Injection: direct injection with steroid results in rapid improvement or resolution of arthritis, with effects lasting 3-24 months. Is rapid and inexpensive.
2 .NSAIDs: Most commonly recommend Naproxen because T1/2allows for BID dosing. Adverse effects are uncommon and usually preventable. Can see dyspepsia/GI ulcer, renal injury, and pseudoporphyria.
3 Systemic Corticosteroids:highly effective for short term management, extremely poor long-term monotherapy. Long list of adverse effects. Will sometimes be used to induce remission while awaiting proper treatment (MTX)
**Please do NOT use without d/w rheumatology, as it will mask/treat joints**
- Methotrexate: Disease modifying anti-rheumatic drug (DMARD). First line therapyfor all extensive JIA or disease refractory to NSAID/injection. Used in low doses (1% of SE cfchemo)– is well tolerated, safe, and efficacious in 80% of patients.
5.Other DMARDS:Leflunomide, Sulfasalazine, Thalidomide, +/- Plaquenil.
What are the general goals of therapy for treatment of JIA? (3)
- what is the first line treatment of JIA?
- what subspecialties (2) should be consulted for all patients diagnosed with JIA?
- Eliminate inflammation AND Prevent joint damage
- Promote normal growth and function
- Minimize medication toxicity
-first line treatment:
NSAIDs
-two consultants:
- Rheumatology
- Ophthamology to rule out uveitis (ESPECIALLY those with oligoarthritis and positive ANA)
What are the most common sites of involvement in enthesitis-related arthritis? (4)
Insertion sites of:
- Quadriceps tendon
- Patellar tendon
- Achilles tendon
- Plantar fascia
N.B. can also see dactylitis, tarsitis
JIA What are the SE of MTX?
Adverse effects include
- immunosuppression (minor degree) + Vaccines: Cannot have LIVE vaccinations per current recommendations
- transaminitis (reversible in vast majority),
- GI intolerance / oral ulcers (manageable).
- Pregnancy: Is extremely teratogenic while using it. Should avoid alcohol.
What is the algorithm for treatment of oligoarticular JIA?
- Try NSAID –> if no remission,
- try intraarticular corticosteroid injection –> if no remission,
- consider methotrexate or other second line agent
What is the algorithm for treatment of polyarticular JIA?
Remember that this is more severe and causes worse functional impairment since it’s multiple joints so you’re more aggressive!
- Try NSAIDs or intraarticular corticosteroid (IAC) injections –> no response –>
- start second line agent right away (methotrexate, leflunomide or sulfasalazine) –> if no response,
3. optimize second line agent and consider
4. IAC or low dose PO steroids as bridging therapy –> if no response, consider
- biologic anti-TNF therapy
***May need to start a second line agent as part of initial therapy for children with severe polyarthritis
What is the algorithm for treatment of systemic JIA?
- Try NSAIDs first OR for severe disease, consider starting corticosteroids or biologic agent (anti-IL-1 or anti-IL-6) right away
- -if tried NSAIDs and no response –> systemic corticosteroids –> if no response –>
- add biologic anti-IL-1 or anti-IL-6 therapy
****note that you DO NOT use methotrexate/sulfasalazine/etc.
What is the definition of oligoarthritis?
-two subcategories?
Definition: arthritis affecting 1-4 joints during first 6 months of disease
- subcategories:
1. Persistent: no more than 4 joints affected throughout disease course
2. Extended: more than 4 joints affected after first 6 months of disease
What is the diagnostic criteria for psoriatic arthritis? -what is the clinical hallmark of this condition?
- Arthritis AND psoriasis OR
- Arthritis with at _least 2 o_f the following:
a. dactylitis (clinical hallmark!!!)
b. nail-pitting or onycholysis
c. psoarisis in a first degree relative
What is the diagnostic criteria for RF-negative-polyarthritis? -what about RF-positive polyarthritis?
RF-negative polyarthritis:
- Arthritis affecting 5 or more joints during first 6 months of disease
- RF testing negative
RF-positive polyarthritis:
- Arthritis affecting 5 or more joints during first 6 months of disease
- 2 or more positive tests for RF at least 3 months apart during first 6 months of disease
What is the most common subtype of JIA?
-most frequent joints involved? (4)
Oligoarthritis
- most frequent joints involved:
1. Knees 2. Ankles 3. Wrists 4. Elbows
Which population is usually affected by RF positive polyarthritis?
Adolescent girls = this is essentially adult-type rheumatoid arthritis presenting in a child or adolescent
-usually involve the PIP and MCP joints
Which population of JIA patients are at highest risk for developing uveitis?
ANA positive oligoarthritis
A 3 yo Hawaiian boy comes to your ED with peeling of fingers and toes. He had 14 days of fever, red eyes, a swollen neck, a red tongue and fussiness. You would like to order an echo but can’t get one for 24 hours. What should you initiate at this time?
LOW DOSE ASPIRIN!!! This is a patient in the convalescent phase of kawasaki disease and thus would not benefit from IVIG or high dose aspirin.
Once they are in the convalescent phase, they need low dose aspirin at 3-5 mg/kg/day.
What are less common clinical manifestations of KD (ie. clinical features other than those in diagnostic criteria)? (3 categories)
1. CNS/Eyes: Irritability (aseptic meningitis)/Uveitis
2. Organs:
a. GIT: Gastroenteritis (abdo pain, vomiting, diarrhea)/Gallbladder hydrops
b. Nephro: Sterile pyuria from urethritis
c. Cardiac: Myocarditis/pericarditis
d. Sytemic: MAS
3. MSK: Arthritis
JD What is the criteria for KD Dx?
Diagnosis: Requires (A) and at least 4/5 (B)
- (A) Fever for > 5 days (4 if IVIG eradicates fever)
- (B) Any 4 of the following: CRASH
C- Conjunctivitis- Bilateralnonpurulentbulbar conjunctivitis (80-90%)
R- Rash- Polymorphous rash (>90%) - nonvesicular(AHA clarifies to maculopapular, erythroderma, or EM like)
A- Adenitis Cervical LN (50%) - at least one node > 1.5 cm.
S- Strawberry tongue: Oropharyngeal changes (80-90%) - red/fissured lips, strawberry tongue, pharyngeal erythema
H - Hands/ Feet changes: Extremity changes (80%) - erythema / edema (acute phase), desquamation (convalescent phase)
What is the diagnostic criteria for: -incomplete KD -atypical KD -alternate way to make diagnosis for KD aside from the 5 or more days of fever + 4/5 features
Incomplete KD:
if 5 or more days of fever with 2-3 features instead of 4 -commonly seen in infants who are at higher risk of coronary artery involvement
Atypical KD:
if KD with unusual manifestation (eg. renal failure)
Alternate way to diagnose KD: in presence of fever AND coronary artery involvement on echo, <4/5 criteria is sufficient
What is the most worrisome complication of KD and when does this occur?
- risk factors for developing complication? (6)
- if untreated, what is the chance of developing this complication?
What does the risk change to if treated?
Coronary artery aneurysms = occur 6-8 wks after acute illness
-risk factors: 1. Males 2. Infants 9 yo 3. Prolonged fever 4. Asian or hispanic ethnicity 5. Lab: Thrombocytopenia or Hyponatremia
-if untreated: 25% will develop coronary artery aneurysms
-if treated (IVIG given within 10 days): decreased to 4%
What is the usual age group for KD? -what is the pathophysiology?
3 mo-5 yo
-small vessel vasculitis with predilection for coronary arteries; thought to be triggered by infectious agent
KD What is the criteria for Incomplete KD Dx?
Diagnosis may be made in the presence of
(A) Fever for > 5 days and
(B) if the following scenario are met:
•Elevated ESR (40) / CRP (30) AND
at least 3 supplementary criteria confirm diagnosis
i) Labs: Anemia, albumin < 31, elevated ALT, sterile pyuria, leukocytosis, thrombocytosis (after day 7)
ii) Echo: Positive echocardiogram (alone) is sufficient
•If ESR/CRP not high enough, clinical observation warranted; if high enough, check labs and treat accordingly.
ALTERNATE DX:
- Incomplete KD is alternatively diagnosed in any patient with fever and any feature, who then develops CA dilatation.
- In infants < 6 months, consider with fever alone.
List rsik factors for DDH (5 F’s and 2 T’s)
Name 5 risk factors for DDH (5 F’s and 2 T’s)
- F:
Frank Breech, Oligohydramnios (uterine)
Female, First born
Family history of DDH
- T: Twins, Tight swaddling
