Rheumatology/MSK Flashcards
List diagnostic criteria of JDM
- Proximal, symmetric muscle weakness
(Neck flexors, shoulder girdle, hip flexors) - Classic rash: Heliotrope rash, Gottrons Papules
- Elevated muscle enzymes
- EMG changes
- Muscle biopsy
Other symptoms/signs: Nailfold capillary changes Fevers Dysphagia Arthritis Muscle tenderness Fatigue
List 2 long term complications of JDM
Lipodystrophy-progressive loss of subcutaneous and visceral fat
Calcinosis
What tests would you order to diagnose JDM?
Diagnosis is clinical!
But if uncertain, order CK, EMG, MRI (T2 fat-suppressed images show inflammation)
Muscle biopsy is last resort!
Treatment of JDM
Steroids
Methotrexate
PT/OT
Avoid sun exposure, sun screen
What is the pathophysiology of periodic fever syndromes?
-Autoinflammatory
-Exaggerated innate immune response
-Excess release of pro-inflammatory cytokines
(especially IL-1β)
What are the different types of periodic fever syndromes?
- FMF
- PFAPA
- TRAPS (TNF receptor associated periodic fever syndrome)
- MVK (Mevalonate kinase deficiency)/HyperIgD
- FCAS (familial cold autoinflammatory syndrome)
- MWS (Muckle Wells)
What are the features of FMF?
Fever 1-3 days duration*** Irregular interval <10 years (80%) Serositis (peritonitis***, pleuritis, pericarditis), Synovitis Erysipelas-like rash Monoarthritis***
What are the features of PFAPA?
Fever 3-5 days duration Regular (every 3-6 wks)*** < 5 years of age in most*** Aphthous ulcers Pharyngitis Cervical adenitis
What are the features of TRAPS?
Fever 1-3 weeks duration*** Irregular interval At least 2-6x per year <10 years in 75% Exercise may trigger*** Abdominal pain Severe myalgia Arthralgia (large joints) Migratory erythematous plaques Periorbital edema Conjunctivitis*** Serositis
What are the features of MVK/HyperIgD syndrome?
3-7 days duration Every 4-6 weeks Triggered by vaccination, minor trauma, stress Onset <1 year in 70%*** Headache Mucosal aphthous Abdominal pain Vomiting Diarrhea Painful adenopathy Arthritis Splenomegaly Polymorphic rashes;
What are the features of FCAS?
1-24 hours duration*** < 1 year of age**** Irregular interval Triggered by cold Migratory urticarial rash Conjunctivitis, Headache Fatigue Myalgia Arthralgia Joint stiffness
What are the features of MWS?
1-3 days duration Variable age of onset Irregular interval Urticaria-like rash*** Arthralgia or non-erosive arthritis Conjunctivitis Progressive sensorineural hearing loss***
What a reasonable initial workup of periodic fevers?
Bloodwork when well and with fever:
CBC with differential (exclude cyclic neutropenia)
ESR
CRP
Blood culture (early on in course)
Other investigations selectively:
Creatinine, liver function, serum Ig, ANA, ANCA, RF
What are the diagnostic criteria for PFAPA?
1) Regularly occurring fever episodes with an early age of onset (<5 years)
2) Constitutional symptoms in the absence of URTI with at least one of the following ▫ Aphthous stomatitis ▫ Cervical adenopathy ▫ Pharyngitis
3) Exclusion of cyclic neutropenia
4) Completely asymptomatic interval between episodes
5) Normal growth and development
What is the treatment for PFAPA?
1) Prednisone single dose at onset of symptoms
2) Consider tonsillectomy
How do you diagnose FMF?
Clinical features
Genetic testing-80% have MEFV mutation (autosomal recessive)
How do you treat FMF and what is the purpose of treatment?
Colchicine
- Reduces attack rate
- Normalizes acute phase reactants
- Prevents amyloidosis)
What is the most common long term complication of untreated FMF?
Amyloidosis (50%)
-Proteinuria–>renal failure
Other complications:
Joint contractures
Abdominal adhesions
In what ethnicities is FMF more common?
Sephardic Jews, Turks, Armenians, Arab
List 4 poor prognostic factors in Kawasaki Disease
- Young age
- Male
- Prolonged fever
- Lab abnormalities →neutrophilia, thrombocytopenia, transaminitis, hyponatremia, hypoalbuminemia, elevated CRP
- Asian, Pacific Islander, Hispanic ethnicities (higher risk of coronary artery abnormalities)
Criteria for Classic Kawasaki Disease
5 days of fever + 4/5 of:
- Bilateral non-exudative bulbar conjunctival injection
- Oral/mucosal changes (strawberry tongue, dry cracked lips, injection of mucosa/pharynx)
- Polymorphous exanthema (anything BUT vesicular)
- Cervical LAD >1.5 cm (nonsuppurative, usually unilateral)
- Extremity changes (palmar/plantar erythema/edema)
What are the 3 clinical phases of KD?
- Acute:
- Febrile phase, usually lasts 1-2 weeks
- Perineal desquamation - Subacute:
- Lasts ~2 weeks
- Fever resolves
- Desquamation (periungual–>entire hand/foot)
- Thrombocytosis
- Development of coronary aneurysms
- Highest risk of sudden death - Convalescent:
- All clinical signs have disappeared
- Lasts until the ESR returns to normal (6-8 weeks after onset)
List 3 clinical features of KD that are not in the diagnostic criteria
Irritability (aseptic meningitis) Meatitis, dysuria, sterile pyuria Oligo/polyarthritis Hepatitis Anterior uveitis Gallbladder hydrops
What percentage of untreated patients with KD develop coronary aneurysms and WHEN?
Untreated 25%
Treated <5%
Typically develop week 2-3 of illness
List 10 laboratory findings consistent with KD
↑WBC, predominantly neutrophils
Normocytic, normochromic anemia
Platelets normal then rapidly ↑↑ after day 7
↑ESR/CRP
Sterile pyuria
↓Na
↓Albumin
↑AST/ALT
↑Bilirubin
CSF pleocytosis
As per AHA guidelines, when should echocardiograms be done in KD (if results are normal)?
At diagnosis
2-3 weeks
6-8 weeks
At 1 year (with lipid profile!)
List 3 conditions in the differential for KD and how do you differentiate them?
Adenovirus-exudative pharyngitis/conjunctivitis
Measles-koplik spots, leukopenia
Scarlet fever-conjunctivitis not common
Toxic shock syndrome-renal insufficiency, coagulopathy, pancytopenia
Drug hypersensitivity reactions-periorbital edema, minimally elevated ESR
SJIA-diffuse LAD and HSM
Treatment of KD
IVIg 2g/kg
Low dose ASA (3-5 mg/kg/day)
(No longer using high dose ASA)
Define IVIg resistant KD
Fever 36 h after IVIG
How do you treat IVIg resistant KD?
2nd dose IVIg
Other options: methylpred
What is the natural history of coronary aneurysms in KD?
50% of coronary aneurysms regress to normal diameter by 1 to 2 years
Giant aneurysms are unlikely to resolve and are most likely to lead to thrombosis or stenosis
Diagnostic criteria for HLH
Need 5/8:
- Fever ≥38.5°C
- Splenomegaly
- Cytopenias: ≥2 of anemia (<100), thrombocytopenia (< 100); ANC <1.0
- Hypertriglyceridemia and/or hypofibrinogenemia
- Hemophagocytosis in bone marrow, spleen, lymph node, or liver
- Low or absent NK cell activity
- Ferritin >500 ng/mL
- Elevated soluble CD25 (soluble IL-2 receptor alpha; reflector of NK fxn)
List the 3 most common rheumatologic causes of MAS
- Systemic JIA
- Lupus
- Kawasaki disease
List clinical features of HSP
Rash (palpable purpura, low extremities) Edema Arthralgias GI symptoms (including intussuseption, perforation) Renal
Others: Orchitis Testicular torsion Carditis Pulmonary hemorrhage
What is acute hemorrhagic edema of infancy?
Isolated cutaneou sleukocytoclastic vasculitis affecting infants < 2 yrs with fever and no other organ involvement
Indication for steroids in HSP
Significant GI symptoms
NOT for renal disease
What % of patients with HSP have recurrence?
30%
Often within 6 months
List laboratory abnormalities in HSP
Leukocytosis Thrombocytosis Anemia ESR/CRP elevated Elevated IgA
What are the diagnostic criteria for JIA (in general)
Onset <16 years of age
Duration >=6 weeks
Exclusion of other causes of arthritis
Xray findings in JIA
Growth acceleration/inhibition Peri-articular osteoporosis Subchrondral erosions Loss of cartilage Eventually bone fusion
List 3 risk factors for uveitis in JIA
+ANA
Girl
Monoarticular arthritis
Onset <6 years
List long term complications associated with JIA
Leg length discrepancy Flexion contractures Scoliosis Delayed motor development Muscle atrophy
List 3 complications of uveitis
Synechiae (can result in small, irregular pupil)
Glaucoma
Cataract
Vision loss
List features of oligoarticular JIA
<=4 joints in 1st 6 months Asymmetric Knee, ankle, elbow Uveitis (30-50%) ANA+ (70%)
List features of polyarticular JIA
>4 joints in 1st 6 months Symmetric Small and large joints Slowly progressive (unless RF +) Uveitis (5%) ANA + 40-50%
Diagnostic criteria for enthesitis related arthritis
Arthritis ± enthesitis with ≥2 of:
- Sacroiliac tenderness or spinal pain
- HLA-B27+
- Arthrithis in male > 6
- Acute anterior uveitis
- FamHx in 1st or 2nd degree relative of confirmed HLA-B27-associated disease
Differential diagnosis of polyarthritis
• Inflammatory
- JIA
- SLE, other connective tissue diseases
- Vasculitis – HSP
- IBD
- Serum sickness
- CRMO
• Infection
- Acute – viral (parvovirus), bacterial (gonococcal)
- Post-infectious – reactive/rheumatic fever
• Malignancy (systemic)
• Mechanical
-Hypermobility, skeletal dysplasia
• Pain syndrome
-Fibromyalgia
Treatment of polyarticular JIA
NSAIDs
Methotrexate
Biologics
Diagnostic criteria for psoriatic arthritis
Arthritis plus 2 of the following
– Nail pits
– Dactylitis
– Family history of psoriasis
Diagnostic criteria for psoriatic arthritis
Arthritis plus 2 of the following
– Nail pits
– Dactylitis
– Family history of psoriasis
Can have uveitis
Diagnostic criteria for systemic JIA
- > 2 weeks spiking fever
- Arthritis + at least one of:
• Rash (evanescant, correlates with fever spikes, salmon-coloured)
• Generalized lymphadenopathy
• Hepatosplenomegaly
• Serositis
List 3 clues that MAS is developing in a child with a rheumatologic disorder
Dropping cell lines Elevated ferritin >1000 Falling fibrinogen Falling ESR Neurologic symptoms
Diagnostic criteria for sJIA
- Fever >2 weeks and daily x 3 days
- Arthritis
- At least one of:
• Rash
• Generalized lymphadenopathy
• Hepatosplenomegaly
• Serositis
What is the classic fever pattern in SJIA?
Quotidian
1-2 spikes/day
Associated with evanescent rash
List 4 organisms that cause reactive arthritis
Chlamydia Shigella Campylobacter Yersinia Giardia Salmonella
How often should you screen patients with JIA with eye exams?
Eye exam q 3-12 months depending on ANA status, age, duration of disease
e.g. ANA+, <6 years, <4 years after diagnosis=every 3months
ANA-, <6 years, <4 years after diagnosis=every 6 months
How often should you screen patients with JIA with eye exams?
Depends on 4 factors:
- Type of JIA
- Age of onset
- Duration of disease
- ANA status
e.g. ANA+, <6 years, <4 years after diagnosis=every 3months
ANA-, <6 years, <4 years after diagnosis=every 6 months
(THIS NIT PICKY STUFF WAS AN OLD QUESTION)
Before starting biolgoic response modifier therapy, what test should be done? (CPS)
TST
When should inactivated vaccines be administered before starting biologics? (CPS)
Inactivated vaccines should be administered 14 or more days before starting BRM
When should inactivated vaccines be administered before starting biologics? (CPS)
Inactivated vaccines should be administered 14 or more days before starting BRM
Should patients receiving biologics get the inactivated influenza vaccine? (CPS)
YES
But can have diminished immune response
When should live vaccines be administered before starting biologics? (CPS)
4 weeks or more before starting BRM
List 3 adverse effects of methotrexate
Cytopenias Oral ulcers Hepatotoxicity Infection Pneumonitis Teratogenic
What percentage of healthy individuals are ANA positive?
20%
More significant if >1:160
List one disease associated with the following antibodies:
i) Anti-dsDNA
ii) Anti-smith antibody
iii) Anti-RNP
iv) APLA
v) Antihistone antibodies
vi) APLA
i) Anti-dsDNA: SLE
ii) Anti-smith antibody: SLE
iii) Anti-RNP: SLE, mixed connective tissue disorder, Raynauds
iv) Anti-Ro/La: sicca, Sjogren, SLE, neonatal lupus
v) Antihistone antibodies: drug induced lupus
vi) APLA: SLE, thrombosis
***specific for SLE
What is one thing you should monitor in patients on hydroxychloroquine?
Retinal toxicity
Need eye exam 6-12 months
Diagnostic criteria for SLE
Diagnosis: >4 of 11 features:
- Malar rash
- Discoid rash
- Serositis
- Oral/nasal ulcers
- Arthritis
- Photosensitivity
- Hematologic (hemolytic anemia, lymphopenia, thrombocytopenia <100)
- Renal (nephritis)
- ANA positive
- Immunologic (autoantibodies, low C3/C4)
- Neuro (seizures, psychosis)
Other features:
- Constitutional sx
- Alopecia
- Raynaud
- LAD, HSM
List 3 differences between idiopathic SLE and drug induced lupus
Drug induced lupus
- Antihistone antibody positive
- Anti-dsDNA negative
- Normal C3/C4
- CNS, renal, hematologic manifestations rare
List medications associated with drug-induced lupus
Minocycline
Anticonvulsants (phenytoin on past exam!)
Sulfonomides
Anti-arrhythmics
List the 3 cardinal systems involved Granulomatosis with polyangiitis (Wegener’s)
- Upper airway
- Sinusitis
- Nasal ulceration
- Saddle nose deformity
- Epistaxis
- Hearing loss - Lower airway
- Cavitary lesions
- Pulmonary nodules
- Pulmonary hemorrhage - Renal
- Nephritis
Other:
- Eye (conjunctivitis, scleritis, uveitis, optic neuritis, orbital pseudotumour)
- Skin (palpable purpura***, ulcers)