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
Acute phase (I)
- time course
- clinical
- lab
- treatment
A
-
time course
- 1-2 wks
-
clinical
- Fever, conjunctivitis, oropharyngeal changes, cervical adenopathy, rash, swollen hands
-
lab
- Increased ESRD & CRP
-
treatment
- HIgh-dose IVIG, high-dose aspirin
18
Q
Subacute phase (II)
- time course
- clinical
- lab
- treatment
A
-
time course
- wks-mo
-
clinical
- Defervescence of inflammation, peeling from nailbeds or distal extremities, coronary artery aneurysms
-
lab
- Decreased ESR & CRP
- Increased platelet count
-
treatment
- Low-dose aspirin
19
Q
Convalescent phase (III)
- time course
- clinical
- lab
- treatment
A
-
time course
- wks-yrs
-
clinical
- Gradual resolution of aneurysms
-
lab
- Normalization of all laboratory findings
-
treatment
- Continue low-dose aspirin only if aneurysms remain
20
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
21
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
22
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)
23
Q
How is JRA classified?
A
- Determined on the basis of clinical features during first 6 mo of disease
- 3 categories of JRA
- Pauciarticular
- Polyarticular
- Systemic
24
Q
Pauciarticular JRA
- definition
- epidemiology
- subtypes
- articular involvement
A
- < 4 joints involved, 40% of cases
- Subtypes
- Early-onset
- Late-onset
- Swelling of 1 or 2 joints, not necessarily symmetric
- Most common joints: knees & hips
25
What is **early-onset** pauciarticular JRA?
* Female predominant
* Present 1-5 YO
* +ANA (75%)
* High risk for chronic uveitis (50%)
* Inflammation of the iris & ciliary body
* Monitor by regular slit-lamp evaluations
26
What is **late-onset** pauciarticular JRA?
* Male predominant
* Present \>8 YO
* Typical patient (like ankylosing spondylitis)
* Male, HLA-B27 positive
* Hips & sacroiliac joints
* Uveitis less common
27
**Polyarticular JRA**
* definition
* subtypes
* articular involvement
* **\>4 joints involved, 40% of cases**
* Systemic involvement & extra-articular features mild or absent
* Subtypes
* RF-negative disease
* RF-positive disease
* **Symmetric polyarthritis**
* Small joints (hands, feet)
* Large joints (knees, ankles, hips)
28
Describe the **subtypes** of Polyarticular JRA
* Based on absence/presence of serum RF
* IgM molecule directed against IgG
* Females \> Males
* **RF negative disease**
* Early & late in childhood
* **RF positive disease**
* Children \>8 YO
* More severe, high risk of severe arthritis & rheumatoid nodules
29
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**
30
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
31
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
32
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
33
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**
34
**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
35
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
36
**CNS** involvement of SLE
* Headache
* Encephalopathy
* Seizures
* Psychosis
* Transverse myelitis
37
**Skin** findings of SLE
* Malar rash
* "butterfly" covering nasal bridge & cheeks
* Photosensitivity
* Alopecia
* Raynaud's phenomenon
38
**Arthralgias & arthritis** of SLE
* Migratory & transient
* Rarely causes joint deformity or erosion
* Myositis
39
**GI** involvement of SLE
* Hepatosplenomegaly
* Splenic infarction
* Mesenteric thrombosis (secondary to vasculitis)
* Sterile peritonitis
40
**CV** involvement of SLE
* Most frequent manifestation: **pericarditis**
* CHF
* Arrhythmias
* Sterile valvular vegetations (Libman-Sacks endocarditis)
* Neonates born to mothers w/ SLE
* **Congenital heart block**
* Secondary to transplacental passage of maternal antibodies
41
**Pulmonary** involvement of SLE
* Pleuritis
* Pulmonary hemorrhage
* Interstitial fibrosis
42
**Renal** involvement of SLE
* Nearly _universal_, lupus nephritis sub-clinical
* Glomerulonephritis
* Nephrotic syndrome
* HTN
* Subsequent renal failure
43
**Hematologic** manifestations of SLE
* Low WBC counts (leukopenia)
* Anemia of chronic disease
* Thrombocytopenia
* Coombs+ hemolytic anemia
44
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
45
What are the **laboratory** findings of SLE?
* Elevated ESR & CRP
* Anemia of chronic disease or anemia secondary to hemolysis
* Leukopenia
* Thrombocytopenia
* Urinalysis may show proteinuria
* Depends on extent of renal disease
* Rheumatologic markers
* **Antiphospholipid Ab** = increased risk of thrombotic events
* **Decreased complement (C3, C4)**
* Active disease
* Immune-complex mediated complement activation
46
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
47
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**
48
What is the **prognosis** of SLE?
* **Survival rate 90% at 5-10 yrs after diagnosis**
* Major causes of mortality
* Infection
* Renal failure or nephritis
* CNS complications
49
**Dermatomyositis**
definition
epidemiology
* **Inflammatory condition of muscle**
* **Progressive muscle weakness w/ characteristic skin findings**
* 5-14 YO, mean age of onset 6 YO
* Female:male = 2:1
50
What are the top 3 clinical features of **Dermatomyositis**?
* Constitutional symptoms
* Fatigue, anorexia, malaise, low-grade fevers, weight loss
* Cutaneous findings (sun-exposed areas)
* Proximal muscle weakness
51
What are the characteristic **cutaneous** findings of Dermatomyositis?
* **Periorbital violaceous heliotrope rash**
* May cross nasal bridge
* **Gottron's papules**
* Skin over MCP/PIP joints
* Erythematous & hypertrophic
52
Describe the **proximal muscle weakness** associated w/ Dermatomyositis
* Hip girdle & legs
* Insidious in onset
* Wks-mo after eruption of skin findings
* **+Gowers' sign**
* Difficulty standing from the sitting position & having to "climb" up the thighs for support
53
What are 6 other common manifestations of Dermatomyositis?
* **Neck flexor muscle weakness**
* **Calcinosis**
* Ca deposition in muscle, fascia & subq
* 40% of children
* **Nail bed telangiectasias**
* **Constipation**
* GI smooth muscle dysfunction
* **Dysphagia**
* **Cardiac involvement**
* Conduction abnormalities
* Dilated CM
54
How is **Dermatomyositis** diagnosed?
* Classic clinical presentation
* Proximal muscle weakness
* Characteristic rashes
* Abnormal electromyography findings
* Abnormal muscle biopsy findings
* Increased muscle enzymes
* Creatinine phosphokinase
* AST, ALT, LDH, aldolase
55
How is Dermatomyositis **managed**?
* Mainstay of therapy: **corticosteroids**
* Other immunosuppressive agents (severe)
* MTX
* Cyclophosphamide
* Cyclosporine
* Vitamin D & Calcium supplementation
* Repair osteopenia
* Decrease fracture frequency
56
What are some **complications** of Dermatomyositis?
* **Aspiration pneumonia**
* Diminished gag reflex
* **Intestinal perforation**
* GI vasculitis
* **Osteopenia**
* Steroid therapy & muscle weakness
* Frequent fractures
57
What is the **prognosis** of Dermatomyositis?
* Children \> adults
* No associated w/ malignancy in pediatric dermatomyositis (25% in adults)
* **Mortality rate ~3%**
58
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
59
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
60
The cause of Rheumatic Fever is \_\_\_\_\_\_.
**Unknown**
\*appears to be autoimmune\*
61
What are the **4 major clinical features** of Rheumatic Fever?
* Cardiac involvement
* Polyarthritis
* Sydenham's chorea
* Skin involvement
62
**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
63
**Polyarthritis** of Rheumatic Fever
* **Migratory, asymmetric, painful**
* 70% of patients
* Elbows, knees, ankles, wrists
* Does _NOT_ result in chronic joint disease
64
**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
65
**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
66
What are the **minor** features of Rheumatic Fever?
* Fever
* Arthralgias
* Leukocytosis
* Increased ESR
* Prolonged PR interval on ECG
67
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
68
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
69
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**
70
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
71
What is **Lyme Disease**?
* Reactive inflammatory disorder of the skin, heart, CNS & connective tissues
* Spirochetal infection w/ *Borrelia burgdorferi*
* Transmitted via tick bite
72
What is the **epidemiology** of Lyme Disease?
* High risk areas reflect the natural habitat of ticks of the *Ixodes* species (vectors)
* Woodlands & fields
* New England states, Pacific coast, Midwest
73
What is the **etiology** of Lyme Disease?
Vector? Organism?
* **Vector**
* Deer tick: *Ixodes scapularis* (US)
* Ixodes pacificus (Western US, less common)
* **Organism**
* *B. burgdorferi*
* Bloodstream of human host while tick engorges itself w/ blood
* Infected tick must be attached _\>36-48 hrs_
74
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
75
What are the stages of Lyme disease?
* **Early disease (1-4 mo)**
* Early localized disease
* Local cutaneous invasion
* Subsequent inflammation
* Early disseminated disease
* Cutaneous findings
* Other organ systems
* **Late disease (5-12 mo)**
* ****7% children
* _Arthritis_
76
**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
77
**Early disseminated** disease of Lyme disease
* Skin
* 25% children
* Multiple secondary **erythema migrans** lesions (smaller than initial lesion)
* Constitutional symptoms
* Neurologic
* **Aseptic meningitis (1%)**
* **Facial nerve palsy (3%)**
* **Encephalitis**
* Carditis (rare)
* **Heart block** or myocarditis
78
How is Lyme disease diagnosed?
Strongly suggested in the presence of the epidemiologic risk factors & classic erythema migrans rash
79
**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
80
Management of Lyme disease
* Aimed at eradicating B. burgdorferi
* Early localized disease/late disease w/ arthritis
* **Doxycycline** (children _\>_9 YO)
* **Amoxicillin**
* Carditis & meningitis
* **IV** **ceftriaxone** or **penicillin**
81
What is the prognosis of Lyme Disease?
* Children treated have **excellent** prognosis
* Recurrent symptoms or chronic sequelae rare
82
What are the **seronegative** spondyloarthropathies?
* Reactive arthritis
* Psoriatic arthritis
* Ankylosing spondylitis
* Arthritis of IBD
83
What is Reactive arthritis?
* Inflammation of the joints triggered by a microorganism
* Enteric or sexually transmitted pathogen
* **Reiter's disease**
* Arthritis, uvethritis, conjunctivitis
* *Chlamydia trachomatis*
84
What is Psoriatic arthritis?
* Arthritis of the small & large joints
* Patients w/ psoriatic skin disease
* Scaly skin plaques, nail pitting, onycholysis (separation of the nail from the nailbed)
85
What is Ankylosing spondylitis?
* **Male-predominant, HLA-B27-related**
* Joints of the LE & axial skeleton
* **Enthesitis**: inflammation of the tendinous insertions on the bone
* High risk: males w/ late-onset pauciarticular JRA
86
What is arthritis of IBD?
* **Ulcerative colitis, Crohn's disease**
* Some patients HLA-B27+
* Involvement of axial skeleton
* Pattern indistinguishable from ankylosing spondylitis
87
What are 3 examples of rheumatologic vasculitides?
* Takayasu's arteritis
* Polyarteritis nodosa
* Wegener's granulomatosis
88
What is Takayasu's arteritis?
* **Large vessel vasculitis**
* Asian female adolescent or young adult
* Constitutional symptoms & aneurysmal dilatation or thrombosis of the aorta, carotid or subclavian arteries
89
What is Polyarteritis nodosa?
* Aneurysms & thrombosis of the small & medium-sized vessels
* Brachial, femora, mesenteric arteries
90
What is Wegener's granulomatosis?
* **Necrotizing granulomas in multiple organs**
* Respiratory tract
* Kidneys
* Constitutional symptoms
* Severe sinusitis
* Hemoptysis
* Glomerulonephritis
91
What is Sjogren's syndrome?
* **Classic triad**
* Sicca syndrome (dry mouth & eyes)
* High titers of autoAb (ANA, RF)
* Connective tissue disease
92
What are the two types of **scleroderma**?
* Systemic scleroderma
* CREST syndrome
93
What is Systemic Scleroderma?
* Systemic sclerosis
* **Excessive fibrosis & subsequent dysfunction of multiple organ systems**
* Skin & vessels of the heart, kidneys, lungs, GI
* _Hallmark_: skin thickening w/ loss of dermal ridges, resembling "tightened" skin
94
What is CREST syndrome?
* **C**alcinosis
* **R**aynaud's phenomenon
* **E**sophageal involvement
* **S**clerosis of the skin
* **T**elangiectasias
