Chapter 26: Rheumatology

Ankylosing Spondylitis - Presentation, Associations, Labs

PRES: Adolescent male with back pain, morning stiffness that gets better with activity.
ASSOCIATIONS: Uveitis, IBD
LABS: HLA-B27 positivity; ANA/RF normal

Dermatomyositis

PRES: Proximal muscle weakness, voice change/difficulty swallowing

Cutaneous Findings of Dermatomyositis

Heliotrope Rash - Violacenous butterfly discoloration of malar region/eyelids
Nail fold telangiectasias
Gottrons Papules - Papules on the DIP/PIP
Extensor surface rash of limbs

Dermatomyositis Lab

Elevated CK

Henoch Schonlein Purpura Pathophysiology

Autoimmune vasculitis associated with IgA deposition in skin, kidney beans, GI tract, joints

HSP Labs

Elevated BUN/Cr
Heme+ Stool
Hematuria/Proteinuria
Platelets and PT/PTT are normal

HSP Presentation

Palpable purpura of lower extremities and buttock, colicky abdominal pain that mirrors intussusception; antecedant viral/bacterial infection, arthritis/arthralgia

Types of JIA

Polyarticular
Oligarticular
Systemic/Stills
Psoriatic
Enthesitis

Oligoartivcular JIA

<5 joints affected
Associated with uveitis when ANA positive

Polyarticular JIA

> 5 joints affected

Systemic JIA/Still's Disease

Systemic manifestations include quotidian fever, leukocytosis, rash, hepatosplenomegaly, lymphadenopathy, pleuritis, pericarditis, serositis

Rash of JIA

Small, salmon colored macules with central clearings, come and go
Koebner Phenomenon - Stroking skin can produce rash

Enesthitis JIA

Some combinatinon of enesthitis, arthrophaty and SI joint tenderness
HLA-B27 positivity common
Unveitis common

Psoriatic JIA

Psoriasis + Arthropathy, uveitis common

JIA Complications

Leg length discrepancy
Joint contracturea
Permanent joint destruction
Blindness from uveitis

JIA Tx

NSAIDs --> Steroids, Immunosuppresives (MTX)

MTX Side Effects

Immunosuppression - Avoid live vaccines
Leukemia/Lymphoma
GI Distress, Pulmonary
Mouth Sores
Neural Tube Defects

Kawasaki Dx

5 days of fever, plus 4 of:
1.5 cm cervical lymphadenopathy
conjunctivitis
oral mucous membrane involvement
extremity changes
rash