case conference
DESCRIPTION
Case Conference. Samatha Madhavarapu. 21 m/o F with limping. HPI. Intermittent limp of R leg started 6 weeks ago. Constant limp since 3 days Worse upon awakening Stiffness in R knee. Transient warmth and redness + Not able to bear weight initially, improves over 2 hrs. - PowerPoint PPT PresentationTRANSCRIPT
![Page 1: Case Conference](https://reader030.vdocument.in/reader030/viewer/2022033103/56812f8d550346895d950bf3/html5/thumbnails/1.jpg)
Samatha Madhavarapu
![Page 2: Case Conference](https://reader030.vdocument.in/reader030/viewer/2022033103/56812f8d550346895d950bf3/html5/thumbnails/2.jpg)
![Page 3: Case Conference](https://reader030.vdocument.in/reader030/viewer/2022033103/56812f8d550346895d950bf3/html5/thumbnails/3.jpg)
HPI• Intermittent limp of R leg started 6 weeks ago.• Constant limp since 3 days• Worse upon awakening• Stiffness in R knee.• Transient warmth and redness +• Not able to bear weight initially, improves over
2 hrs.• Was outdoors in Upstate at BBQ 8 weeks ago• No fever, rash, recent URI• No trauma, diarrhea,
![Page 4: Case Conference](https://reader030.vdocument.in/reader030/viewer/2022033103/56812f8d550346895d950bf3/html5/thumbnails/4.jpg)
HPI Contd…PMH: NoneFH: grand father has seizure disorder. No
bone/joint problems Immunizations: UTDNKDAMeds: Tylenol PRN pain.Labs done 6 weeks ago: wbc7.6, 33.9/66.3,
ESR 18, xray R knee Normal.
![Page 5: Case Conference](https://reader030.vdocument.in/reader030/viewer/2022033103/56812f8d550346895d950bf3/html5/thumbnails/5.jpg)
Physical ExamVital signs:HEENT : NormalHeart , Lungs ,Abdomen: NormalSkin: No rashR knee: decreased extension, no swelling, no
redness, no warmth.Other Jts: FROM.
![Page 6: Case Conference](https://reader030.vdocument.in/reader030/viewer/2022033103/56812f8d550346895d950bf3/html5/thumbnails/6.jpg)
LABSBMP: 136/4.9/ 101/22/12/0.4/139/10.5Total Protein/ Albumin: 7.5/4.7LFT: 0.2/01, 38/19, 244CBC: 7.3/ 12/36.3/392ESR: 15
![Page 7: Case Conference](https://reader030.vdocument.in/reader030/viewer/2022033103/56812f8d550346895d950bf3/html5/thumbnails/7.jpg)
Other LabsCRP: 0.2ANA negativeLyme Ab titre: 1.2Anti CCP antibody: 8.3HLA B 27: NegativeRF: 20.0Ultrasound of Knees: Small R knee jt effusion
![Page 8: Case Conference](https://reader030.vdocument.in/reader030/viewer/2022033103/56812f8d550346895d950bf3/html5/thumbnails/8.jpg)
DD of Arthritis in ChildrenRheumatic &
Inflammatory Diseases
Seronegative Spondyloarthropathies
Infectious IllnessesReactive ArthritisImmunodeficiencies
Congenital & Metabolic Disorders
Bone & Cartilage Disorders
Neoplastic DisordersHematologic
DisordersPain Syndromes
![Page 9: Case Conference](https://reader030.vdocument.in/reader030/viewer/2022033103/56812f8d550346895d950bf3/html5/thumbnails/9.jpg)
Juvenile Rheumatoid ArthritisJRA most common rheumatic disease of
childhoodSynovitis of peripheral joints manifested as
swellingJRA is not a single disease, but a category of
diseases.
![Page 10: Case Conference](https://reader030.vdocument.in/reader030/viewer/2022033103/56812f8d550346895d950bf3/html5/thumbnails/10.jpg)
Criteria for Classification of JRA Age of onset: < 16 yrs Arthritis in > or = 1 joint Duration of the Disease: > or = 6 weeks Onset type is defined by type of articular
involvement in the first 6 months after onset:
Poly arthritis: > or = 5 inflamed joints Oligo arthritis: < or = 4 inflamed joints Systemic Disease: arthritis with intermittent
fever
![Page 11: Case Conference](https://reader030.vdocument.in/reader030/viewer/2022033103/56812f8d550346895d950bf3/html5/thumbnails/11.jpg)
Classification of Chronic Arthritis in Children
ACR, ELAR & ILAR classificationOnly ACR criteria have been
statistically validated.
![Page 12: Case Conference](https://reader030.vdocument.in/reader030/viewer/2022033103/56812f8d550346895d950bf3/html5/thumbnails/12.jpg)
Characteristic ACR ELAR ILAR
Onset Types 3 6 6
Age of onset of arthritis < 16 yr <16 yr <16 yr
Duration of arthritis >= 6 wk
>= 3mn
>= 6wk
Juvenile Ankylosing Spondylitis
Doesn’t include
includes
Includes
Juvenile Psoriatic Arthritis
Doesn’t include
includes
includes
Inflammatory Bowel Disease
Doesn’t include
includes
includes
Exclusion of other diseases
YES YES YES
![Page 13: Case Conference](https://reader030.vdocument.in/reader030/viewer/2022033103/56812f8d550346895d950bf3/html5/thumbnails/13.jpg)
Etiology• Unknown• Immunogenetic Susceptibility (Specific HLA
subtypes)• External Triggers - Viruses( EBV, Parvo virus B19, Rubella) - Hyperreactivity to self antigens(type 2
collagen) - Enhanced T-cell reactivity to heat schock
proteins of bacteria.
![Page 14: Case Conference](https://reader030.vdocument.in/reader030/viewer/2022033103/56812f8d550346895d950bf3/html5/thumbnails/14.jpg)
Epidemiology Incidence of JRA: 13.9/100,000 Sex: Pauci and poly articular disease more in girls Systemic onset –equal frequency in boys and girlsRace: Prevalnce of JIA lower in Urban African –
American compared to Caucasians Oligo 40% newly diagnosed cases in Caucasians. Blacks with JRA were older and less likely to test
positive for ANA or to have uveitis, more likely to test positive for Ig M RF
![Page 15: Case Conference](https://reader030.vdocument.in/reader030/viewer/2022033103/56812f8d550346895d950bf3/html5/thumbnails/15.jpg)
Pathogenesis• Synovitis: Villous hypertrophy & edema of
subsynovial tissues.• Vascular endothelial hyperplasia• Infiltration of mononuclear and plasma cells.• Pannus formation with erosion of cartilage and
bone.• Recriutment of T-cells specific to synovial non
specific antigens., facilitated by specific HLA types.
![Page 16: Case Conference](https://reader030.vdocument.in/reader030/viewer/2022033103/56812f8d550346895d950bf3/html5/thumbnails/16.jpg)
Clinical FeaturesOnset insidious or abruptMorning stiffness and gellingEasy fatiguabilityJoint pain (not very severe) and swelling,
limited joint movt, not erythematous.May have preceding illness
![Page 17: Case Conference](https://reader030.vdocument.in/reader030/viewer/2022033103/56812f8d550346895d950bf3/html5/thumbnails/17.jpg)
Oligoarthritis/Pauciarticular• Affects 4 or fewer joints• Typically larger joints (knees, ankles, wrists).• Starts with 1 joint• Monoarticular involvement of hip, upper
extremity large joints never presenting sign in JRA.
• If knee is affected-limping+, esp morning• Chronically- atrophy of extensor muscles of thigh,
tight hamstrings & knee flexion contractures.• Associated with HLA-DR8
![Page 18: Case Conference](https://reader030.vdocument.in/reader030/viewer/2022033103/56812f8d550346895d950bf3/html5/thumbnails/18.jpg)
Polyarthritis/Polyarticular• Minimum 5 joints should be effected.• Both large & small jts of upper and lower
extremities• Resembles adult RA and HLA profile.• Associated with HLA –DR4• Rheumatoid nodules in severe form• Micrognathia- chronic TM joint disease• C-Spine involvement- atlantoaxial
subluxation
![Page 19: Case Conference](https://reader030.vdocument.in/reader030/viewer/2022033103/56812f8d550346895d950bf3/html5/thumbnails/19.jpg)
Systemic Onset• Arthritis with visceral involvement• Characteristic intermittent spiking fevers to
>/= 39c for >/= 2 weeks. • Febrile episode assoc with evenescent (< 1
hr) macular rash, linear or circular, 2-5 mm, over trunk & proximal extremities.
• Arthralgia, myalgia• Hepatosplenomegaly,Lymaphadenopathy• Serositis/pericardial effusion• Photophobia (uveitis), irregular iris due to
synechiae
![Page 20: Case Conference](https://reader030.vdocument.in/reader030/viewer/2022033103/56812f8d550346895d950bf3/html5/thumbnails/20.jpg)
![Page 21: Case Conference](https://reader030.vdocument.in/reader030/viewer/2022033103/56812f8d550346895d950bf3/html5/thumbnails/21.jpg)
LabsCBC with diff:
Lymphopenia,Thrombocytosis,microcytic anemia. Neutropenia is uncommon.
ESR: - Always elevated with systemic JRA. - Usually elevated with polyarticular but
within reference range in pauci articular. - When elevated, ESR helps to monitor
success of medical treatment
![Page 22: Case Conference](https://reader030.vdocument.in/reader030/viewer/2022033103/56812f8d550346895d950bf3/html5/thumbnails/22.jpg)
Labs contdANA:Positive in 40-85% with oligo/poly articularUnusual with systemic onsetTitres do not correlate with disease
severity.Associated with incraesed risk of uveitisRF: Rare in systemic JRA. Marker of persistence of polyarticular JRA
into adulthood., devpt of rheumatoid nodules and poor functioning.
![Page 23: Case Conference](https://reader030.vdocument.in/reader030/viewer/2022033103/56812f8d550346895d950bf3/html5/thumbnails/23.jpg)
Total protein and albumin: levels are often decreased during active disease
ALT test: to exclude hepatitis (viral or autoimmune) prior to starting NSAIDS
U/A: to r/o infection (trigger of JRA or transient postinfectious arthritis) and nephritis (seen in pts with SLE)
![Page 24: Case Conference](https://reader030.vdocument.in/reader030/viewer/2022033103/56812f8d550346895d950bf3/html5/thumbnails/24.jpg)
ImagingX-ray: When 1 jt is affected , to r/o osteomyelitis or
septic arthritis. Soft tissue swelling, regional osteoporosis,
osteopenia, sub chondral bone erosions, narrowing of cartilage spaces, fusion of nueral arches.
MRI synovial inflammation, early minimal changes seen
Echo cardiography: Serositis
DEXA: osteopenia
![Page 25: Case Conference](https://reader030.vdocument.in/reader030/viewer/2022033103/56812f8d550346895d950bf3/html5/thumbnails/25.jpg)
X rays
![Page 26: Case Conference](https://reader030.vdocument.in/reader030/viewer/2022033103/56812f8d550346895d950bf3/html5/thumbnails/26.jpg)
ManagementMultidisciplinary Team for care of childrenMain goal: maximize daily functioning ,
minimize drug toxicity.Key predictor of long term outcome is early
diagnosis and referral to rheumatology team.Diet: Include 3 servings of calcium rich foodsActivity: more active, better the prognosis
![Page 27: Case Conference](https://reader030.vdocument.in/reader030/viewer/2022033103/56812f8d550346895d950bf3/html5/thumbnails/27.jpg)
NSAIDS• Used to treat all subtypes of JRA (40-60%
children show improvement).• Mean duration for anti-inflammatory effect in
JRA- 30 days• Most with pauci and few with poly respond to
NSAID alone . • Rofecoxib , celecoxib (selective Cox-2 inhibitors)
~ similar to naproxen effectiveness• Adverse : nausea, decresead appetite, abd pain.
Less gastritis Naproxen induced pseudoporphyria
![Page 28: Case Conference](https://reader030.vdocument.in/reader030/viewer/2022033103/56812f8d550346895d950bf3/html5/thumbnails/28.jpg)
Methotrexate• Safest, most efficacious, least toxic of of 2nd
line agents for JRA.• Used in 60% patients with poly JRA• Inhibits DHFR, purine synthesis. • Pts unresponsive to PO MTX benefit from SC
or IM administration. • Well tolerated and less toxicity in children.• Pts who respond well have improved growth,
functionality, radiographic improvement.
![Page 29: Case Conference](https://reader030.vdocument.in/reader030/viewer/2022033103/56812f8d550346895d950bf3/html5/thumbnails/29.jpg)
• For overwhelmingly inflammatory or systemic illness.• Bridge therapy for those who did not respond to conventional therapy• Ocular control of uveitis (drops or injections)• Intra articular use :initial therapy in pts with
only 1 or 2 joint involvement• Improvement in symptoms in 2-3 days,
which last for at least 6 mo in 60% and 1yr in 45%
Glucocorticoids
![Page 30: Case Conference](https://reader030.vdocument.in/reader030/viewer/2022033103/56812f8d550346895d950bf3/html5/thumbnails/30.jpg)
Anti TNF alpha • Etanercept,: Only one approved for children• Fusion protein with TNF receptor monomer to Fc portion of Ig G1.• Administered SC twice weekly used in active
polyarticular JRA who fail MXT therapy. • JRA assoc-chronic uveitis that is
inadequately responsive to steroid therapy.• Not to be used with h/o chronic infections• R/o TB before starting rx
![Page 31: Case Conference](https://reader030.vdocument.in/reader030/viewer/2022033103/56812f8d550346895d950bf3/html5/thumbnails/31.jpg)
SulphasalazineImproved joint inflammation & labs
compared to placebo.GI irritation and rash.Steven johnson in pts with active systemic
JRA.CI in porphyria and G6PD deficiency
![Page 32: Case Conference](https://reader030.vdocument.in/reader030/viewer/2022033103/56812f8d550346895d950bf3/html5/thumbnails/32.jpg)
Prognostic features• Child with oligo: esp. girls, onset< 6yrs age
–chronic uveitis risk• Polyarticular: RF, rheumatoid nodules• Systemic onset: number of joints involved,
duration of inflammation, severity of arthritis.
• Limb length discrepancy, contractures,• Disability continues into adulthood in 20%• Chronic pain syndromes.• Psychological complications.
![Page 33: Case Conference](https://reader030.vdocument.in/reader030/viewer/2022033103/56812f8d550346895d950bf3/html5/thumbnails/33.jpg)
http://www.youtube.com/watch?v=NqyB-cTxvs8
![Page 34: Case Conference](https://reader030.vdocument.in/reader030/viewer/2022033103/56812f8d550346895d950bf3/html5/thumbnails/34.jpg)
![Page 35: Case Conference](https://reader030.vdocument.in/reader030/viewer/2022033103/56812f8d550346895d950bf3/html5/thumbnails/35.jpg)
![Page 36: Case Conference](https://reader030.vdocument.in/reader030/viewer/2022033103/56812f8d550346895d950bf3/html5/thumbnails/36.jpg)
![Page 37: Case Conference](https://reader030.vdocument.in/reader030/viewer/2022033103/56812f8d550346895d950bf3/html5/thumbnails/37.jpg)
![Page 38: Case Conference](https://reader030.vdocument.in/reader030/viewer/2022033103/56812f8d550346895d950bf3/html5/thumbnails/38.jpg)