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Flashcards in Rheumatology Deck (48)
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1

Henoch-Schonlein Purpura

Definition

Epidemiology 

  • Systemic IgA-mediated vasculitis involving the skin, joints, GI tract & kidneys
  • Disease of young children
    • 75% <10 YO 
    • Median age of onset: 5 YO
    • Male:female = 2:1 

2

What is the prodrome of Henoch-Schonlein Purpura?

  • Viral syndrome or URI 
  • 20% comitant or prior group A ß-hemolytic stretococcal infection 

3

What are the skin manifestations of Henoch-Schonlein Purpura?

  • Urticarial or erythematous maculopapular lesions progress to petechiae & palpable purpuric lesions concentrated on the buttocks & LE
  • Children: edema of hands, feet, scrotum, scalp
  • Infants: facial rash + edema 
  • GI & joint symptoms may precede diagnostic rash by days/wks (30%)

4

What are the joint manifestations of Henoch-Schonlein Purpura?

  • Occur in 80% of patients 
  • Arthralgia or arthritis 
  • Most involved: knees & ankles 

5

What are the GI manifestations of Henoch-Schonlein Purpura?

  • 67% of patients
  • Colicky abdominal pain 
  • GI bleeding (occult blood or bloody stool)
  • Increased risk of intussusception 

6

What are the renal manifestations of Henoch-Schonlein Purpura?

  • Wide range of presentation
    • Mild hematuria & trace proteinuria 
    • Gross hematuria
    • Nephrotic syndrome
    • Chronic renal insufficiency 
    • ESRD (1%)
  • May not become clinically apparent for up to 3 mo after initial presentation in 25% of nephritis patients 

7

How is Henoch Schonlein Purpura diagnosed

  • History & characteristic physical exam
  • Routine lab tests are not specific/diagnostic 
  • Increased serum IgA levels (50%)
  • Circulating IgA immune complexes in serum & IgA deposition in skin & glomeruli 
  • Platelet counts normal despite petechiae & purpura (skin rash is a nonthrombycytopenic purpura)

8

Henoch Schonlein Purpura

management 

prognosis 

  • Relief of symptoms (pain control & hydration)
  • Steroids for abdominal pain & arthritis 
  • Prognosis
    • Recover w/i 4 wks 
    • Recurs at least once in 50%
    • Long-term morbidity depends on severity of nephritis 

9

What is Kawasaki Disease (Mucocutaneous Lymph Node Syndrome)?

  • Acute febrile vasculitis of childhood 
  • Unknown origin 
  • Multiple organ systems
    • Heart
    • Skin
    • Mucous membranes
    • GI tract
    • CNS
    • Joints
    • Peripheral vascular bed 

10

What is the epidemiology of Kawasaki disease?

(sex, ethnicity, age)

  • The most common cause of acquired heart disease in the US
  • Male:female = 3:2
  • Children of Asian ethnicity 
  • Mean age: 18-24 mo 
  • 80% cases in children <5 YO 

11

What is the diagnostic criteria for Kawasaki Disease?

  • Fever >102oF (38.9oC) lasting >5 days
  • 4 of the following:
    • Bilateral conjunctivitis: bulbar injection w/ limbic sparing w/o exudate
    • Oropharyngeal changes: pharyngitis, strawberry tongue, red/cracked/swollen lips
    • Cervical adenopathy: unilateral nonsuppurative cervical LN >1.5 cm diameter
    • Rash: truncal polymorphous rash: erythematous maculopapular, morbilliform, scarlatiniform 
    • Changes in distal extremities
      • Early (first 7-10 days): brawny edema & induration of the hands/feet w/ erythematous palms & soles
      • Later (7-10 days after fever): peeling around nail beds or distal extremities
  • The illness must not be explainable by any other disease process 
    • Bacterial, viral, rickettsial infections
    • Rheum conditions (JRA)
    • Drug rxns 

12

What are 6 other clinical features of Kawasaki Disease?

  1. CV manifestations
  2. Urethritis (sterile pyuria)
  3. Aseptic meningitis 
  4. Hydrops of the gallbladder (10%, RUQ pain)
  5. Arthritis (sterile) or arthralgias 
  6. Anterior uveitis 

13

What are the cardiovascular manifestations of Kawasaki Disease?

  • Coronary artery aneurysms (20%)
    • Untreated patients
    • Subacute phase (days 7-14)
  • Low grade myocarditis (common)
  • CHF
  • Arrhythmias 
  • Aneurysms of brachial arteries 

14

What is the time course of Kawasaki Disease?

Triphasic

  • Phase I: acute phase (1-2 wks)
  • Phase II: subacute phase (wks-mo)
  • Phase III: convalescent phase (wks-yrs)

15

What are the laboratory findings of Kawasaki Disease?

  • No laboratory tests are pathognomonic 
  • Acute phase
    • Increased ESR & CRP
  • Subacute phase
    • Increased platelet count
    • Decreasing ESR & CRP
  • Convalescent phase
    • Lab findings normalize w/i 6-8 wks 

16

What are the options for managing Kawasaki Disease?

  • Anti-inflammatory therapy, serial ECGs
  • IVIG
    • High dose (2 g/kg) IVIG + ASA
    • Initiated w/i 10 days of fever onset 
    • Decrases prevalence of coronary artery dilatation & aneurysms detected 2-7 wks later 
  • ASA
    • Acute phase: high-dose ASA (anti-inflammatory)
    • Subacute phase: low-dose ASA (anti-platelet) 
  • Steroids
    • Controversial; increased morbidity 
    • Patients unresponsive to IVIG 

17

What is the prognosis of Kawasaki disease?

  • If CAD absent, no long-term sequelae
  • Even if CAD present, mortality <1% & aneurysms commonly regress 
  • Long-term prognosis unclear, increased risk of atherosclerotic heart disease in adulthood possible 

18

What is the definition of Juvenile Rheumatoid Arthritis?

Disorder characterized by chronic joint inflammation in children, w/ or w/o extra-articular involvement 

19

What is the epidemiology of JRA?

(age, sex)

  • Most common pediatric rheumatic disease w/ arthritis as the distinguishing manifestation 
  • Mean age of onset: 1-3 YO (<6 mo unusual)
  • Most commonly in females, exceptions:
    • Males equally likely to have systemic onset JRA
    • Males more likely to have late-onset pauciarticular JRA (M:F = 10:1)

20

What is Systemic-onset JRA (Still's disease)?

What are the top 4 clinical symptoms?

  • 20% of cases, severe > joint 
  • Clinical
    • High spiking fevers
      • >39oC (102.2oF)
      • Late afternoon/evening
      • Return quickly to baseline/normal
      • Diff: "fever of unknown origin"
    • Transient salmon-colored rash
      • Trunk & proximal extremities 
      • During febrile episodes 
      • Evanescent (w/ fevers then fades)
      • Non-pruritic 
    • Hepatosplenomegaly 
    • Lymphadenopathy 

21

What are some additional clinical features of Still's disease

  • Fatigue, anorexia, weight loss, failure to thrive 
  • Serositis (pericarditis, pleuritis) 
  • CNS involvement (meningitis, encephalopathy) 
  • Myositis, tenosynovitis 

22

What is the diagnostic criteria for JRA?

  • Age of onset <16 YO
  • Arthritis in >1 joint defined as:
    • Swelling or effusion *OR*
    • Limitation of motion, tenderness, increased warmth 
  • Duration of disease >6 wks 
  • Exclusion of other causes of arthritis 

Systemic JIA causes inflammation in one or more joints for 6 weeks’ duration in a child younger than 16 years of age and is often accompanied by a high fever (103° F [39.4° C] or higher) that lasts at least 2 weeks, fatigue, inflammation of joints, and a skin rash. Pericarditis, pleuritis, anemia, or enlargement of lymph nodes, liver or the spleen may also occur. About 10 percent of children with JIA will have the systemic form.

23

What are the laboratory findings of JRA?

  • Non-specific, reflect existence/extent of inflammation 
  • Anemia
    • Microcytic & hypochromic
    • Anemia of chronic disease 
  • Elevated acute-phase reactants 
    • ESR, CRP, platelet count 
  • Rheumatoid markers
    • RF negative in a majority of patients 
    • ANA
      • 75% of early pauci JRA,
      • 50% of polyarticular JRA
      • NOT present in Still's/late onset pauci 

24

How is JRA managed? 

 

  • Control of inflammation 
    • NSAIDs ease pain & inflammation
    • Immunomodulation for severe symptoms
    • Glucocorticoids, MTX, sulfasalazine, hydroxychloroquine 
  • Mechanical & physical measures
    • PT/OT, selective splinting to minimize joint contractures 
  • Surgery
    • Recalcitrant joint contractures/destruction
  • Psychosocial support 

25

Systemic Lupus Erythematosus (SLE)

  • definition
  • epidemiology
  • etiology 

  • Multisystem autoimmune disorder 
    • Widespread inflammation of the connective tissues
    • Immune complex-mediated vasculitis 
  • Female:Male = 8:1
  • Age of onset rare before 10 YO, peaks in adolescence 
  • Cause unknown 
    • Triggers?: drug rxns, excessive sun exposure, infections, hormone changes 

26

What are the 9 clinical features of SLE?

  1. Constitutional symptoms
    1. Fever, weight loss, malaise 
  2. CNS 
  3. Skin findings
  4. Arthralgias & arthritis 
  5. GI involvement 
  6. CV involvement 
  7. Pulmonary involvement 
  8. Renal involvement 
  9. Heme manifestations 

Symptoms include fatigue, skin rashes, fever and joint involvement with non-deforming arthritis with effusion and tenderness. SLE can involve many organ systems (kidney, skin, blood cells and nervous system). A malar rash across the cheeks and bridge of the nose in the shape of a butterfly is seen in 95% of SLE cases. 

 

27

What is the diagnostic criteria for SLE?

SOAP BRAIN MD

  • Serositis (pleuritis or pericardial inflammation)
  • Oral or nasal mucocutaneous ulcerations 
  • Arthritis, non-erosive 
  • Photosensitivity 
  • Blood cytopenias 
    • Leukopenia, hemolytic anemia, thrombocytopenia
  • Renal disease (hematuria, proteinuria, HTN)
  • ANA+
  • Immunoserology abnormalities 
    • dsDNA Ab, anti-Smith An, false+ RPR or VDRL assays 
  • Neuro symptoms
    • Encephalopathy, seizures, psychosis 
  • Malar rash (butterfly rash)
  • Discoid lupus 

28

What are the rheumatologic markers of SLE?

  • ANA 
    • Universally elevated (>95%)
    • Non-specific 
  • RF
    • Elevated, non-specific 
  • Anti-dsDNA Ab
    • Specific (only in SLE)
    • Used as markers for active disease, especially nephritis 
  • Anti-Smith Ab 
    • Less prevalent, but very specific 
    • Can't be used as measure of disease activity 

29

How is SLE managed?

  • Control of inflammation
    • NSAIDs - myalgias & arthralgias
    • Immunosuppressive meds
      • Glucocorticoids: mainstay of therapy; low-dose oral or high-dose IV pulse
      • Cyclophosphamide: IV pulse; severe lupus nephritis; adverse effects include infertility & gonadal failure, secondary malignancies, hemorrhagic cystitis 
      • Azathioprine, MTX, cyclosporine 
  • Treatment of complications 
    • Thrombosis & anti-phospholipid Ab: anticoagulation w/ LMWH or warfarin
    • Renal failure: dialysis, fluid & electrolyte management, renal transplant 
  • Psychosocial & family support

30

What is the definition of Rheumatic Fever

  • Delayed, nonsuppurative, autoimmune complication of URI w/ group A ß-hemolytic streptococcus (Streptococcus pyogenes)
  • Inflammation of the connective tissues 
  • Heart, BV, joints, CNS, skin