Rheumatology Flashcards
1
Q
Henoch-Schonlein Purpura
Definition
Epidemiology
A
- 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
Q
What is the prodrome of Henoch-Schonlein Purpura?
A
- Viral syndrome or URI
- 20% comitant or prior group A ß-hemolytic stretococcal infection
3
Q
What are the skin manifestations of Henoch-Schonlein Purpura?
A
- 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
Q
What are the joint manifestations of Henoch-Schonlein Purpura?
A
- Occur in 80% of patients
- Arthralgia or arthritis
- Most involved: knees & ankles
5
Q
What are the GI manifestations of Henoch-Schonlein Purpura?
A
- 67% of patients
- Colicky abdominal pain
- GI bleeding (occult blood or bloody stool)
- Increased risk of intussusception
6
Q
What are the renal manifestations of Henoch-Schonlein Purpura?
A
-
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
Q
How is Henoch Schonlein Purpura diagnosed?
A
- 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
Q
Henoch Schonlein Purpura
management
prognosis
A
- 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
Q
What is Kawasaki Disease (Mucocutaneous Lymph Node Syndrome)?
A
- Acute febrile vasculitis of childhood
- Unknown origin
- Multiple organ systems
- Heart
- Skin
- Mucous membranes
- GI tract
- CNS
- Joints
- Peripheral vascular bed
10
Q
What is the epidemiology of Kawasaki disease?
(sex, ethnicity, age)
A
- 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
Q
What is the diagnostic criteria for Kawasaki Disease?
A
- 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
Q
What are 6 other clinical features of Kawasaki Disease?
A
- CV manifestations
- Urethritis (sterile pyuria)
- Aseptic meningitis
- Hydrops of the gallbladder (10%, RUQ pain)
- Arthritis (sterile) or arthralgias
- Anterior uveitis
13
Q
What are the cardiovascular manifestations of Kawasaki Disease?
A
-
Coronary artery aneurysms (20%)
- Untreated patients
- Subacute phase (days 7-14)
- Low grade myocarditis (common)
- CHF
- Arrhythmias
- Aneurysms of brachial arteries
14
Q
What is the time course of Kawasaki Disease?
A
Triphasic
- Phase I: acute phase (1-2 wks)
- Phase II: subacute phase (wks-mo)
- Phase III: convalescent phase (wks-yrs)
15
Q
What are the laboratory findings of Kawasaki Disease?
A
- 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
Q
What are the options for managing Kawasaki Disease?
A
- 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
Q
What is the prognosis of Kawasaki disease?
A
- 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
Q
What is the definition of Juvenile Rheumatoid Arthritis?
A
Disorder characterized by chronic joint inflammation in children, w/ or w/o extra-articular involvement
19
Q
What is the epidemiology of JRA?
(age, sex)
A
- 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
Q
What is Systemic-onset JRA (Still’s disease)?
What are the top 4 clinical symptoms?
A
- 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
-
High spiking fevers
21
Q
What are some additional clinical features of Still’s disease?
A
- Fatigue, anorexia, weight loss, failure to thrive
- Serositis (pericarditis, pleuritis)
- CNS involvement (meningitis, encephalopathy)
- Myositis, tenosynovitis
22
Q
What is the diagnostic criteria for JRA?
A
- 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
Q
What are the laboratory findings of JRA?
A
- 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
Q
How is JRA managed?
A
-
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
## Footnote
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**
* **S**erositis (pleuritis or pericardial inflammation)
* **O**ral or nasal mucocutaneous ulcerations
* **A**rthritis, non-erosive
* **P**hotosensitivity
* **B**lood cytopenias
* Leukopenia, hemolytic anemia, thrombocytopenia
* **R**enal disease (hematuria, proteinuria, HTN)
* **A**NA+
* **I**mmunoserology abnormalities
* dsDNA Ab, anti-Smith An, false+ RPR or VDRL assays
* **N**euro symptoms
* Encephalopathy, seizures, psychosis
* **M**alar rash (butterfly rash)
* **D**iscoid 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
31
Rheumatic fever epidemiology
* Rare in the US & Western Europe
* Worldwide problem in 1960s
* Children _5-15 YO_
* No gender predilection
* Major risk factor
* **Pharyngitis caused by GABHS**
* Strep strains that cause skin infection (impetigo) DON'T cause RF
32
What are the **4 major clinical features** of Rheumatic Fever?
* Cardiac involvement
* Polyarthritis
* Sydenham's chorea
* Skin involvement
33
**Cardiac** involvement of Rheumatic Fever
* 50% of patients
* _Hallmark_ & most important complication
* **Endocarditis**
* Most common cardiac finding
* Insufficiency of L sided valves (mitral/aortic)
* **Myocarditis**
* Tachycardia out of proportion to extent of fever, cardiac dilatation, HF
* **Pericarditis**
* Pericardial effusions, less common
34
**Polyarthritis** of Rheumatic Fever
* **Migratory, asymmetric, painful**
* 70% of patients
* Elbows, knees, ankles, wrists
* Does _NOT_ result in chronic joint disease
35
**Sydenham's chorea** of Rheumatic Fever
* Occurs later, begins subtly, months after GABHS pharyngitis
* Reflects involvement of the **basal ganglia** & **caudate nuclei**
* Hand clumbsiness --\> choreoathetoid movements w/ emotional lability
36
**Skin involvement** of Rheumatic Fever
* **Erythema marginatum**
* _Nonpruritic rash, pink to red macules_
* Coalesce & spread centripetally w/ central clearing over the trunk & proximal limbs
* **Subcutaneous nodules**
* Rarely seen, associated w/ severe cardiac involvement
* _Small, mobile, painless nodules_
* Bony prominences of the extensor surfaces of the extremities
37
What are the **minor** features of Rheumatic Fever?
* Fever
* Arthralgias
* Leukocytosis
* Increased ESR
* Prolonged PR interval on ECG
38
How is Rheumatic Fever **diagnosed**?
What is the **Jones Criteria**?
* Evidence of recent strep infection & 2 major criteria or 1 major + 2 minor criteria
* **Major criteria**
* Migratory polyarthritis
* Carditis
* Sydenham's chorea
* Erythema marginatum
* Subcutaneous nodules
* **Minor criteria**
* Fever
* Arthralgia
* Previous rheumatic fever
* Leukocytosis
* Elevated ESR
* Elevated CRP
* Prolonged PR interval on ECG
39
What are the **laboratory** findings of Rheumatic Fever?
* Nonspecific inflammatory markers
* **Elevated ESR & CRP**
* **Elevated WBC count**
* Serologic markers
* **Anti-streptolysin-O titers**
* Abnormally elevated (70-80%)
* Evidence of recent GABHS infection
* **Anti-DNase & anti-hyaluronidase Ab**
* Document GABHS infection
* **ECG shows evidence of carditis**
* Decreased ventricular function
* Valvular insufficiency
* Pericardial effusion
40
Management of Rheumatic Fever
What drugs are used?
Supportive therapy?
* Eradication of GABHS infection
* Benzathine **penicillin** IM (one dose)
* Penicillin orally for 10 days
* Control of inflammation
* **NSAIDs** for joint pain/swelling
* Only after diagnosis of RF certain
* **Corticosteroids** for severe cardiac
* CHF, severe valvular dysfunction
* Supportive therapy
* **CHF** - diuretics, dietary salt restriction, digoxin, bed rest
* Sydenham's chorea - **haloperidol**
* Long-term management
* Continuous antimicrobial **prophylaxis**
41
What is the **prognosis** of Rheumatic Fever?
* No chronic sequelae of the joint, skin & CNS manifestations
* **Cardiac inflammation**
* Severe valvular dysfunction: intervention immediately or yrs later
* Valvular insufficiency/stenosis: delayed (\>3 yrs after RF), valve replacement or valvuloplasty
42
What is **Lyme Disease**?
* Reactive inflammatory disorder of the skin, heart, CNS & connective tissues
* Spirochetal infection w/ *Borrelia burgdorferi*
* Transmitted via tick bite
* Infected tick must be attached \>36-48 hrs
43
Clinical features of Lyme disease are caused by \_\_\_\_\_\_\_\_\_\_, and in later stages by \_\_\_\_\_\_\_.
* **Invasion** of B. burgdorferi into local & distant tissues
* Systemic **inflammatory** response against the spirochete
44
**Early localized** disease of Lyme disease
* **Erythema migrans**
* 2/3 of patients
* Annular & "targetlike" rash
* Variable degrees of central clearing
* Asymptomatic, pruritic, painful
* May expand \>12 in diameter
* **Constitutional symptoms**
* Fever, headache, myalgias, fatigue, arthralgias, lymphadenopathy
45
How is Lyme disease diagnosed?
Strongly suggested in the presence of the epidemiologic risk factors & classic erythema migrans rash
46
**Laboratory** diagnosis of Lyme disease
* Measurement of Ab to *B. burgdorferi*
* Serologic testing
* **ELISA** - high sensitivity
* **Western blot** - confirm ELISA
* Other lab tests offer no advantage
47
What is the prognosis of Lyme Disease?
tx
* Children treated have **excellent** prognosis
* Recurrent symptoms or chronic sequelae rare
Doxycycline \>7
amoxicillin \< 7
48
Dermatomyositis
is an autoimmune myopathic illness affecting the pediatric population. **Proximal muscle weakness** in addition to **thick scaling on extensor surfaces**, known as **_Gottron papules_**, are characteristic of dermatomyositis. Results of laboratory studies are often normal at the time of diagnosis. The standard of definitive diagnosis is through muscle biopsy and management is through rheumatology. Without adequate treatment, muscle weakness will often continue to progress. Early recognition of Gottron papules is key to the diagnosis of dermatomyositis.
