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Flashcards in rheum2 Deck (145):
1

some sclerodermal signs

diffuse tight skin
hand contractors
bibasilar or diffuse lung crackles from lung scarring (may be assoc. w. CHF-->pulm. edema, alveoli "popping" back open
or if sounds more like velcro-->dry from fibrosis)

2

sclerodermal CXR

chronic basilar interstitial changes (scarring rather than CHF)

3

sclerodermal labs

Cr may be elevated
UA w. RBCs+

4

systemic scleroderma may be caused by

gabalinium for MRI

5

don't nec. tx...

localized scleroderma

6

diffuse syst. scleroderma

is worst, need to tx
involves trunk, UE, face, neck
"Mouse head" tight mouth
hard time eating/swallowing
swollen head
lungs, heart, kidney, GI, vasc
lung* most common cause of mortality, used to be kidney but can now tx

7

scleroderma affects skin and/or

alimentary tract (fibrosis)
may not have skin pres.

8

diffuse syst. scleroderma GI

GERD, stenosis of LES, lose ability to keep contents in stomach, diff. motility
eso. erosion

9

GAVE gastro antral vascular ectasia

linear rings across gastric mucosa

10

akinetic GIT leads to

bacterial overgrowth

11

pulmonary disease

pulm. HTN, scarring
scleroderma is a vasculopathy: slow ischemia to lungs-->reactive fibrosis, obl. capillary beds
drop in DLCO in PFTs

12

cardiac disfunction

myo/pericardial fibrosis

13

renal involvement

HTN, anemia, hypoperfusion
CKD
RAS activated

14

musculoskeletal involvement

tendon friction rub* - "catching" with palpation
joint contractures
myositis - musc. die off
inflammatory synovitis- hot, red, stiff

15

CREST syndrome

Calcinosis ("cutis" Ca deposits in skin, ulcer-appearance, tophi)
Raynaud phenomenon (tx w/ Ca channel blocker)
Esophageal dysmotility (GERD, lack of peristalsis, just "drip down", chronic ulcers-->gangrene)
Sclerodactyly (contractures of hand, skin tightening(puffy, shiny, red), flexure, inflamm-->fibrosis)
Telangiectasia (cheeks)
-break down of melanocytes

16

linear morphea

straight line over dermatome, scarring, not nec. need tx, limited rxn

17

Mousecof?

puffy face, difficult to open mouth-->need nutr. consult, esp. if diff. swallowing

18

scleroderma lab findings

Mild anemia
Proteinuria
ANA nearly always positive, frequently high
*anti-SCL-70*, Ab against topoisomerase 1 in 1/3 diffuse and 20% CREST
*anti-centromere Ab* in 50% CREST(1% diffuse), highly specific for CREST

19

scleroderma renal crisis

diffuse scleroderma (not CREST)
HTN (>200), proteinuria, AKI,CKI
20% affected
highest mortality originally (now tx w. ACE inhib.)
many go on dialysis (vasculopathy affecting afferent renal arteries-->hypoperf.-->"revving up aldosterone/RAS))

20

scleroderma tx

Treatment of symptoms, no cure
Raynaud’s: CaChBlock, AngRecB’s, sildenafil
Esophagus/GI: PPI’s, small meals, abx for bacterial overgrowth and motility
*Avoid prednisone, can trigger SRC*
(IMARDS)Cyclophospamide, micophenolate mofetil, sildenafil for lung involvement/pulmonary htn

21

pulmonary HTN

is highest mortality in diffuse systemic scleroderma
-live about 5 yrs
(CREST doesn't develop this)

22

inflammatory myopathies

polymyositis
dermatomyositis
inclusion body myosities

23

dermatomyositis tx

prednisone, methotrexate

24

dermatomyositis classic pres.

proximal muscle weakness and a V neck rash
Gottren’s papules present over MCP’s and PIP’s

25

dermatomyositis labs

CPK>1000 implies musc. breakdown
ESR: high
WBC: high
Hgb: low

26

ddx myopathies

Inflammatory Myopathy
Hypothyroidism
Drug-induced myopathy (statins)
HIV and other infections
ALS
Myesthenia Gravis
Muscular Dystrophy
Inherited Metabolic Myopathies

27

dermatomyositis/polymyositis

Prevalence rates of 4-10/million/year

F:M predominance of 2:1

Peak incidence in 2nd decade for PM

Bimodal pattern for DM, peaks during childhood and between ages of 50

28

DM or PM?

musc. wkness
DM: charac. skin manifest
DM: Ca (bimodal prev.)
diff immunopathogenesis

29

DM immunopathogenesis

Humorally-mediated
Infiltrate located around blood vessels
Vasculopathy involving complement
Inflammatory infiltrate of CD4+ T cells and dendritic cells
Abnormal muscle fibers limited to one portion
-more systemic than PM

30

PM immunopathogen

Direct T-cell mediated muscle injury
Infiltrate within the fascicle invading individual muscle fibers (insidious)
No signs of vasculopathy or immune complex deposition
CD8+ T cells appear to recognize Ag on surface of muscle fibers

31

clinical manifestations of myopathies

insidious onset musc. wkness
-symmetric proximal musc.
-distal in late disease
-pharyng/resp. musc
myalgias and tenderness (25-50%)
musc. atrophy in sev., long standing cases

32

dermatologic manifestations myopathies (DM)

May precede onset of myositis mod-yrs
Can be most active component of DM and refractory to therapy
*Gottron’s papules* are pathognomonic feature
Heliotrope rash, Shawl sign, Mechanic’s hands, and nailfold abnormalities – all highly characteristic
Raynauds Phenomena in 25% (vasculopathy)

33

Gottron's papules

Erythematous, scaly eruptions occurring symmetrically over MCPs and IP joints, also on extensor surfaces
Pathognomonic for DM
(vs. SLE)

34

heliotrope rxn

purplish reaction (over eye) with DM

35

Shawl sign

photosn rash in DM

36

DM can effect face like

SLE, like "butterfly rash" not as sev.

37

DM pts can dev. ??? in hand joints

calcinosis

38

DM pts can dev. ??? due to vascular changes

linear excoriations

39

pulmonary manifest. in myopathies

Major cause of morbidity and mortality
Occur primarily or secondarily due to muscle weakness
Hypoventilation (22%) (CO2 retention)
Aspiration Pneumonia (17%)
Frequently present with dysphagia, striated muscles of pharynx and upper esophagus
Interstitial Lung Disease (10-45%)

40

Interstitial lung disease (myopathies)

Interstitial infiltrates and fibrosis (destr. of alveoli)
May occur before, concomitantly, or after onset of skin and muscle disease
Restrictive impairment on PFTs with reduced lung capacities and reduced DLCO
High resolution CT
ILD with DM less responsive to steroid therapy

41

myopathies: cardiac manifestations

Frequency in myositis 6-75%

Cause of death in 10-20%

Arrythmias, conduction abnormalities, myocarditis, pericarditis, secondary fibrosis

Elevation in CK-MB due to inflamed skeletal muscle, involvement of myocardium, or mostly commonly to regenerating muscle

42

misc. myopathy manifests.

Fever

Weight loss

Raynauds

Nonerosive inflammatory polyarthritis
Wrists, knees, small joints of hands
Responsive to treatment of myopathy

43

Antisynthetase Syndrome (myopathies)

30% pts:
Acute onset of disease
Constitutional symptoms
Raynauds
Mechanics hands
Arthritis
*Interstitial Lung Disease
*Anti-synthetase antibodies (Anti-Jo 1)

44

amyopathic DM

don't worry
skin manifest, no musc

45

malignancy w. myopathies

Immune reaction initially directed at tumor cells
crosses over leading to development of myositis
(Myositis autoantigens)
5-7 fold increase in cancer incidence with DM, pk 2 years before or after myopathy dx

46

malignancies with myopathies: adenocarcinomas

cervix
lung
*ovaries*
pancreas, bladder, and stomach (70%)
Nasopharyngeal in SE Asians

47

Ca does not affect..

severity or duration of weakness, CK elevation, or extramuscular manifestations
Less likely to have myositis-specific autoantibodies
Inflammatory myopathy responds to treatment of underlying malignancy
survey for 1st 2-3 post-dx (exc. ovarian ca)

48

myopathy/malignancy dx

Muscle enzymes
Autoantibodies
EMG
Tissue biopsy
MRI Imaging

49

musc. enzyme markers myopathies
prognostic?

CK, aldolase
LD, AST/ALT
** treatment should be guided at patient’s strength, not concentration of muscle enzymes

50

more myopathy dx

ANA (+) in 80%
Anti Ro/La/RNP to rule out underlying CTD

Myositis-specific antibodies (30%)

51

Ab for mechanic hands, Raynauds, interstitial lung disease

Anti-Jo 1:
Ab against anti-histidyl tRNA synthetase

52

myopathy dx: EMG

Abnormal in majority, normal in 11%

Not diagnostic but useful in differentiating myopathy from neuropathic disorders

May help direct site of muscle biopsy

53

myopathy dx: MRI

Areas of inflammation and edema with active myositis, fibrosis, and calcifications
Reveals areas of increased T2 signal for biopsy selection
Useful to monitor response to therapy

54

myopathy dx: musc. biopsy

Definitive test
Quadricep or deltoid contralateral to muscle found to be abnormal on EMG
leukocytes invading musc.

55

PM musc. biopsy

inflamm. cells invade healthy musc-->which become rounded and variable in size

56

DM musc. biopsy

atrophy of fibers near fasc. border
inflamm. cells conc. around BVs (form cuffs) and at border, fiber shrinkage

57

inclusion body myositis biopsy

musc. fiber w/ vacuoles and inclusion bodies
inflamm. cells btw fibers

58

skin biopsy myopathies

may be non-sp.
DM skin findings (Gottron’s papules, Shawl sign, erythroderma)
-may avoid musc. biopsy if charac. pres.

59

myopathies: worse outcome if:

> 6 months delay in treatment after symptom onset
Profound muscle weakness, respiratory weakness
Dysphagia
ILD or cardiac involvement
Malignancy (don't want to dec. IR)

60

these factors do NOT predict disease course/tx response

Gender, race, presence of rash, CK elevation

61

myopathy morbidities

5 year survival was 52-65% in 1970-80’s

Improved to 75-95% from 2001-2006

MC causes of death malignancy, infections, respiratory failure, cardiovascular disease

62

tx response myopathies

PM patients less responsive to glucocorticoids alone than patients with DM or overlap syndromes
50% PM patients refractory to glucocorticoids
87% DM patients responded initially to steroids but 92% flared when tapered
89-100% response rate in patients with overlap myositis

63

goals of myopathy tx

Improve muscle strength

Avoid development of extramuscular complications

Resolution of cutaneous manifestations in DM

64

myopathy tx

Initiation of high doses of Prednisone (1mg/kg/day) for 4-6 weeks for disease control
Pulse dose methylprednisolone if severely ill
Slow taper to lowest effective dose for total duration of therapy 9-12 months or longer

65

myopathy tx monitor

every few wks
Quadriceps/Hip flexors
Deltoids/Neck flexors
*Muscle strength-reliable indicator (vs. enzymes)
Enzymes levels decline within 2 weeks, longer for normalization
**Adjusting Prednisone dosage to normalize muscle enzymes may lead to overtreatment

66

why glucocorticoids fail in myopathy tx

Alternate diagnosis
Glucocorticoid-induced myopathy
Unrecognized malignancy

67

glucocorticoid sparing agents

after 4-6 wks if no resp. to glucocorticoids
Combination therapy for those with ILD or esophageal dysfunction
Azathioprine – GI, BM, Liver
Methotrexate – Stomatitis, GI, Leukopenia
Antimalarials:
Hydroxychlorquine
Effective in 75% in controlling skin disease
No benefit for muscle disease

68

myopathy tx course

No studies addressing optimal duration of therapy
Tapering of Prednisone and Azathioprine/MTX based on clinical improvement, remission, and flares

69

other myopathy tx consids.

Exercise
Aspiration precautions
Avoidance of sunlight
Osteoporosis prevention
Opportunistic infections

70

refractory myopathy tx

(flares)
Add or modify dose of Prednisone
Add, change, or modify dose of Azathioprine/MTX

71

recurrent myopathy tx

Rituximab
IVIG
Cyclosporine/Tacrolimus
Mycophenolate Mofetil
Cyclophosphamide

72

inclusion body myositis

Commonly misdiagnosed as PM
Many “refractory” PM cases are IBM
Make up 15-28% of inflammatory myopathies
Prevalence ranges from 4.9-70 cases/million
M>F, older ppl
distal musc. often normal Cr biopsy
oval, punched out portions of musc.
poor resp. to tx

73

IBM clin features

Proximal and Distal Muscles
Quadriceps, forearm flexors, foot drop
Asymmetric distribution **
Dysphagia (30-60%)
May be presenting symptom
May have Peripheral Neuropathy
Slowly progressive disease

74

IBM dx

Lab Findings
CK normal/mildly elevated,

75

IBM MRI

Inflammatory changes throughout muscle in IBM vs along fascial planes in PM

Abnormalities localized to anterior muscles with more distal and asymmetric involvement

Fatty infiltration and atrophy

76

IBM musc. biopsy

Definitive test for diagnosis and differentiation
CD8+ T cell infiltration
Basophilic rimmed vacuoles
Eosinophilic inclusions
Filamentous inclusions and vacuoles on EM
** Definitive diagnostic feature
Bind antibodies with affinity for beta amyloid

77

IBM px/tx

Rapid loss of strength and function with older age of onset
Respiratory failure and infection causes of death
Poor response to immunosuppressives and glucocorticoids (only mild improvement, CK declined-no prediction on clinical benefit)
Gender, age, duration do not predict response

78

spondyloarthropathy presentation

acutely ill, limp
synovitis, effusion
swollen/tender Achilles tendon (enthesitis)
tenderness along plantar fascia
elevated synovial fluid WBC
elev. ESR, CRP

79

spondyloarthropathies

Ankylosing spondylitis
Psoriatic Arthritis
Enteropathic Arthritis (Crohn’s/UC)
Reactive Arthritis (formerly Reiter’s Syndrome)
Undifferentiated

80

spondyloarthropathy common features

Inflammation of axial joints (SI joints)
Asymmetric oligoarthritis
Dactylitis
Enthesitis
Genital/skin lesions
Eye/bowel involvement
Association with infectious disorders

81

spondyloarthropathy genetic marker
dx?

HLA B27 association
not dx, inclusion factor

82

spondyloarthropathy prevalence

0.5 to 2 percent in Caucasian population

Ankylosing spondylitis and undifferentiated types most common

Reactive arthritis least common

83

spondyarth. muscskel features

Inflammatory back pain
Most common symptom at onset (70%)
Inflammatory vs. Mechanical back pain
Onset

84

spondyarth: peripheral arthritis

Acute onset, affects lower extremities (knees,ankles)
Asymmetrical, affecting 1-3 joints
Joint swelling
enthesitis: Achilles tendon

85

enthesitis

Inflammation at site of insertion of ligaments, tendons
Very specific for spondyloarthritis
commonly seen at insertion of Achilles tendon with swelling or at insertion of plantar fascia ligament into calcaneus
Iliac crest, greater trochanter, epicondyles, tibia, costochondral junctions, occiput, spinous processes

86

dactylitis

“sausage toe/finger”
Differentiated from synovitis by swelling of entire digit
Nonspecific finding
Also seen in TB, syphillis, sarcoid, sickle cell disease

87

spondlyarth.: inflamm. eye disease

Conjunctivitis, anterior uveitis (iritis)
Transient with resolution in several weeks to months
Initial attack acute and unilateral
Episodes do not always parallel disease course

88

spondylarth: inflamm. of bowel mucosa

2/3 of SpA patients have inflammatory lesions
May be acute resembling bacterial enterocolitis
Chronic resembling Crohn’s disease
20% of patients with IBD have signs/symptoms of SpA

89

SpA lab testing

acute phase reactants
**test for bac inf. (G/C) and HIV** C.diff
HLA B27:
Positive in 90% of AS, 70% of undifferentiated SpA
Not diagnostic by itself
Useful in supporting diagnosis in absence of sacroiliitis and ruling out

90

SpA imaging

SI joints: (starts here-sacroilitis)
erosions
Sclerosing
Pseudowidening or narrowing of joint space
Partial or total ankylosing
"jig-saw" like: inflamm-->jagged facets
"bamboo spondyloarthritis": lose kyphosis/lordosis, spinal mobility
lumbar vertebrae lose form

91

ankylosing spondylitis

Chronic inflammatory disease of axial skeleton
Insidious onset of back pain and progressive spine stiffness
May involve peripheral joints and organs
Onset 20-30 years
Prevalence depending on ethnicity
Caucasians > Hispanics > African Americans
5-6% in HLA B27 positive population
No gender difference
Increased risk 5.6-16 fold if 1st degree relative with AS (10-20% if HLA B27)

92

clinical features ank. spond

Low back pain (> 3 months)
Buttock pain, hip pain and shoulder pain, TMJ
Peripheral arthritis
Acute, nonerosive, nondeforming monoarticular involvement
Limited spinal mobility and chest expansion
Flexion of neck, thoracic kyphosis, loss of lumbar lordosis
Enthesitis, Dactylitis
Constitutional symptoms
Fatigue most common

93

ank. spond dx: spinal mobility

limited
Modified Schober’s Test:
Mark L5 spinous process, mark 10 cm above and 5cm below
Measure distance between 2 marks when bending forward
Increase of 5 cm if normal mobility
Occiput to wall test:
Increased distance indicates increased thoracic kyphosis and loss of cervical/lumbar lordosis

94

ank. spond dx: chest expansion

limited
Measured at level of 4th intercostal space
arms elevated and hands folded behind head
Change of

95

ank. spond dx: hip involvement

Flexion deformity poor prognostic indicator
May be masked by compensatory motion in spine
Have patient lie supine and maximally flex one hip
Flexion deformity of contralateral hip if contralateral knee raises up

96

ank. spond dx misc.

SI joint tenderness
Peripheral joint involvement
Enthesitis/Dactylitis

97

ank spond responds to...

NSAIDS (bad SEs)
Marked improvement in 24 to 48 hours

98

ank spond imaging

Sacroillitis
Squaring of vertebral bodies on lateral views in early disease
Syndesmophytes, ankylosing of facet joints, calcification of anterior longitudinal ligaments
Bamboo spine: calcification bridges disks

99

ank spond: extraarticular manifests.

Acute Anterior Uveitis
Unilateral (25-40%)
50% with acute recurrent uveitis have SpA
Pain, photophobia, blurred vision
Treated with local steroids and atropine
Cataracts, Glaucoma


100

ank spond: extraarticular manifests: bone

Osteopenia/Osteoporosis
Falsely high values on bone density testing due to ankylosing and syndesmophytes

101

ank spond risk considerations

Fracture of ankylosed spine
Atlantoaxial subluxation
Cervical cord compression
Most common at C5-C6
Cauda equina syndrome
Bowel inflammation
Cardiovascular disease, AR, Aneurysms
Pulmonary fibrosis and restriction in chest expansion
IgA nephropathy and secondary amyloidosis

102

ank spond px

Hip involvement
Dactylitis
Onset 30)
Limitation of lumbar spinal motion
Poor efficacy of NSAIDS

Male gender, smoking, history of uveitis

103

ank spond tx

NSAIDS
1st line therapy; 70-80% response rate (but CV mort)
Analgesics
Sulfasalazine
For peripheral joint involvement
TNF agents
Infliximab, Etanercept, Adalimumab
80% response rate in first 6 wks of treatment
Improvement of bone density, prevention of uveitis
Costly, no long term data
Physical therapy

104

psoriatic arthritis

Incidence of 6/100,000/year

Prevalence 1-2/1000; 4-30% in patient with psoriasis

Asymmetric joint pain, swelling, and stiffness

Affects DIPs and spine

Less tender than other inflammatory arthritis  joint deformities without much pain

105

psoriatic arthritis pres.

Distal involvement of DIPs (vs RA-NOT DIPs!)

Asymmetric oligoarthritis

106

PA clin features

Skin lesions
Usually precede development of arthritis
Arthritis may precede skin lesions in up to 17%
Nail pitting and onycholysis
80-90% vs. 45% in uncomplicated psoriasis
Severity correlates with severity of skin and joint disease
Pitting edema
Uveitis
Dactylitis
Enthesitis

107

PA dx

Elevated ESR

Rheumatoid factor
Positive in 2-10% of uncomplicated psoriasis and psoriatic arthritis

Anti-CCP
8-16% *but mostly RA*

Antinuclear Antibodies
Low titers (1:40) in up to 50%

108

"pencil in cup" deformity

seen in psoriatic arthritis

109

PA tx

NSAIDS
1st line, control mild inflammatory features
DMARDS
Methotrexate
Skin and joint features
Antimalarials
Cyclosporine
TNF agents
Skin and joints

** none shown to prevent progression of disease

110

Enteropathic Arthritis
-not hit much here

Extraintestinal manifestion of IBD
Occurs in 9-53% of Crohn’s/UC patients
Affects males and females equally

Arthritis affecting spine, SI joints
Spondylitis in up to 26%, males >females
Pain and stiffness in back worse in AM or after rest
Sacroiliitis more common with Crohn’s

111

reactive arthritis triad

postinfectious arthritis, urethritis, conjunctivitis

112

react. arth pathogens (enteric/genital)

Chlamydia trachomatis
Yersinia
Salmonella
Shigella
Campylobacter
C. difficile
*Get G/C, HIV testing*
*if hosp. check for C. diff*
HIV inf

113

react. arthritis

Interval of several days to weeks between preceding infection and onset of arthritis

Asymmetrical mono or oligoarthritis, predominately lower extremities

Prevalence 30-40/100,000; incidence 5-28/100,000/year

114

react arth clin features

Asymmetric oligoarthritis
Enthesitis
Dactylitis

115

react arth clin features: extra-art. symps

Dysuria, pelvic pain
Diarrhea
Conjunctivitis
Oral ulcers
Skin and genital lesions
Nail changes

116

blennorrhagica

pustular lesions on feet w/ reactive arthritis

117

circinate balanitis

lesion on penis in reactive arthritis

118

react arth dx

Urine, stool, and genital cultures

PCR for Chlamydia

Serologies for Yersinia, Salmonella, Campylobacter

Synovial aspiration and fluid analysis

HLA B27 seen in less that 50%

119

react arth px

Most have complete resolution 6 months after presentation
Chronic persistent arthritis in small proportion

120

react arth tx

NSAIDS
Symptomatic relief; do not shorten disease course
Intraarticular steroids
Systemic glucocorticoids
DMARDS-rarely
Sulfasalazine if NSAIDS/steroids not effective

121

react arth tx for sev. cases/infection

TNF agents
Indicated for chronic synovitis if contraindication or refractory to Sulfasalazine
Etanercept, Infliximab, Adalimumab

Antibiotics
Indicated for acute infections not to treat arthritis
Indicated for Chlamydia infections; may prevent development of arthritis
Not recommended for uncomplicated enteric infections
No evidence to support long term antibiotic use in reactive arthritis

122

sarcoidosis mediastinoscopy w/ paratracheal LN biopsy

Fragments of lymph node tissue with multiple well-defined *noncaseating granulomas* with rare microcalcifications
Negative stains for acid fast and fungal microorganisms
No evidence of malignancy

123

sarcoidosis MRI b/l Thigh

Extensive myositis involving bilateral adductor brevis, aductor longus, adductor magnus and sartorius muscles
No intramuscular abscess
No evidence of OM

124

sarcoidosis EMG

peripheral neuropathy, myopathy

125

sarcoidosis

Incidence and Prevalence
United States
Blacks affected more than whites 3:1 to 17:1
Blacks peak incidence in 4th decade of life
Women more susceptible than men
2nd most common cause of lung disease in young adults
Age 20-40, with 2nd peak >60

126

sarcoid etiology

*Unknown cause
Exaggerated inflammatory immune response
Infectious
Mycobacteria spp
M. tuberculosis katG Ab, heat-shock protein 70 Ab, mycolyl trasferase Ag 85A Ab
Propionibacterium acnes
Cryptococcus spp
Occupational exposure or environmental
Berylium
insecticides
Dust
Autoantigen

127

sarcoid patho

Hallmark: *noncaseating granulomas* "hurricane of cellular debris", giant cell formation-no necrosis in center
Local accumulation of inflammatory cells
CD4+ T Cells
Activated monocytes

128

sarcoid genetics

HLA (DR17, DRB1*1101)
HLA-DQB1*0201 (Lofgren’s Syndrome)
BTLN-2 gene on chromosome 6p
Negative costimulatory molecule
don't worry

129

sarcoid symps

Nonspecific
Fever
Sweats
Weakness
Weight loss
Aches and Pains
Psychological Issues
ORGAN SPECIFIC SYMPTOMS

130

sarcoid pulm involv

Cough, Dyspnea, Chest Pain
>90% Lung involvement
PFTs
Restrictive Pattern
DLCO
Reduced Lung Volumes
Obstructive Pattern
Reduced FEV1/FVC ratio
Pulmonary HTN
5% of patients
70% in end-stage fibrosis

131

sarcoid testing

CXR
PFTs
CT scan

132

sarcoid skin

>1/3 of patients

Classic lesions
Erythema nodosum
Maculopapular lesions
Hyper- and hypopigmentation
Keloid formation
Subcutaneous nodules

133

sarcoid: lupus pernio

Bridge of nose
Beneath eyes
Cheeks

Plaque-like induration
Violaceous discoloration
Erode to cartilage and bone

134

sarcoid: Lofgren's syndrome

B/L hilar adenopathy
Erythema nodosum
Arthritis (ankles)
Uveitis

135

sarcoid: eye

Anterior Uveitis
65%
Posterior Uveitis
25%
Retinitis
Pars planitis

Slit lamp
fundoscopic

136

sarcoid: liver

Granulomatous disease in >50% of patients using bx
20-30% have abnormal LFTs
Elevated alkaline phosphate level
Elevated transaminases
Elevated bilirubin in advanced disease

5% have symptoms requiring therapy

137

sarcoid: cardiac

Granulomas in cardiac muscle
Arrhythmias
Cardiomyopathy
Heart block if AV node infiltrated

Mortality due to VT

Testing
EKG
24h ambulatory monitoring
AICD
PET Scan, Cardiac MRI
EP study

138

sarcoid: metabolic

Calcium Metabolism
Hypercalcemia 10% of patients
Hypercalciuria 40% renal calculi 10%
Measure 24h urinary excretion of calcium
More common in whites and men
Increased production of 1,25-dihydroxyvitamin D
Increased intestinal absorption of calcium

renal:

139

chronic sarcoid

Nondeforming arthritis with granulomatous synovitis
Jaccoud's type deformity (nonerosive joint deformity)
Joint swelling adjacent to a sarcoid bone lesion
Dactylitis (sausage-like swelling of one or more digits)
Acute and chronic gouty arthritis, which can be seen in association with sarcoid

140

sarcoid: nervous system

5-10% of sarcoid patients have neurologic symptoms
Central or peripheral nervous system involvement
Cranial mononeuropathy
Facial nerve palsy
HEREFORDT'S SYNDROME: facial palsy, fever, uveitis, enlargement of the parotid gland
Optic neuropathy
Hypothalamic inflammation
Polyuria, disturbances to thirst, sleep, appetite, temperature, libido
Thyroid, gonadal, or adrenal abnormalities

141

sarcoid: NS 2

Granulomatous inflammation of brain
Partial or generalized seizures
Restricted or generalized encephalopathy
Cognitive or behavioral problems
Focal deficits

Spinal cord (perivascular lesions)
Myelopathy or radiculopathy

142

sarcoid: NS 3

Meningeal involvement
Acute aseptic meningitis
Chronic meningitis
CSF: lymphocytic pattern, mild increase in protein, normal to low glucose, oligoclonal bands, elevated ACEs levels

Peripheral
Mononeuropathy
Mononeuritis multiplex
Autonomic and motor polyneuropathies
EMG: axonal neuropathy

143

aggressive syst. sarcoid tx factors

Active neurologic (except Bell’s Palsy)
Cardiac involvement
Severe hypercalemia
Occular disease refractory to topical therapy
Lupus pernio
Bulky LAD, symptomatic splenomegaly
Significant hepatic involvement

144

sarcoid tx

PREDNISONE
First-line therapy
Starting dose 20mg/day
Monitor closely

Acute disease may require IV therapy

In pulmonary involvement, improvement in symptoms, chest radiographs, and PFTs are typical

Failure to respond within 3-4 months suggests steroid refractory disease

145

sarcoid 2nd line tx

Methotrexate
Azathioprine (Imuran)
Leflunomide (+/- MTX)
Mycophenolate
Hydroxycholoroquine