rheum2 Flashcards
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)
sclerodermal CXR
chronic basilar interstitial changes (scarring rather than CHF)
sclerodermal labs
Cr may be elevated
UA w. RBCs+
systemic scleroderma may be caused by
gabalinium for MRI
don’t nec. tx…
localized scleroderma
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
scleroderma affects skin and/or
alimentary tract (fibrosis) may not have skin pres.
diffuse syst. scleroderma GI
GERD, stenosis of LES, lose ability to keep contents in stomach, diff. motility
eso. erosion
GAVE gastro antral vascular ectasia
linear rings across gastric mucosa
akinetic GIT leads to
bacterial overgrowth
pulmonary disease
pulm. HTN, scarring
scleroderma is a vasculopathy: slow ischemia to lungs–>reactive fibrosis, obl. capillary beds
drop in DLCO in PFTs
cardiac disfunction
myo/pericardial fibrosis
renal involvement
HTN, anemia, hypoperfusion
CKD
RAS activated
musculoskeletal involvement
tendon friction rub* - “catching” with palpation
joint contractures
myositis - musc. die off
inflammatory synovitis- hot, red, stiff
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
linear morphea
straight line over dermatome, scarring, not nec. need tx, limited rxn
Mousecof?
puffy face, difficult to open mouth–>need nutr. consult, esp. if diff. swallowing
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
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))
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
pulmonary HTN
is highest mortality in diffuse systemic scleroderma
-live about 5 yrs
(CREST doesn’t develop this)
inflammatory myopathies
polymyositis
dermatomyositis
inclusion body myosities
dermatomyositis tx
prednisone, methotrexate
dermatomyositis classic pres.
proximal muscle weakness and a V neck rash
Gottren’s papules present over MCP’s and PIP’s
dermatomyositis labs
CPK>1000 implies musc. breakdown
ESR: high
WBC: high
Hgb: low
ddx myopathies
Inflammatory Myopathy Hypothyroidism Drug-induced myopathy (statins) HIV and other infections ALS Myesthenia Gravis Muscular Dystrophy Inherited Metabolic Myopathies
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
DM or PM?
musc. wkness
DM: charac. skin manifest
DM: Ca (bimodal prev.)
diff immunopathogenesis
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
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
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
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)
Gottron’s papules
Erythematous, scaly eruptions occurring symmetrically over MCPs and IP joints, also on extensor surfaces
Pathognomonic for DM
(vs. SLE)
heliotrope rxn
purplish reaction (over eye) with DM
Shawl sign
photosn rash in DM
DM can effect face like
SLE, like “butterfly rash” not as sev.
DM pts can dev. ??? in hand joints
calcinosis
DM pts can dev. ??? due to vascular changes
linear excoriations
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%)
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
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
misc. myopathy manifests.
Fever
Weight loss
Raynauds
Nonerosive inflammatory polyarthritis
Wrists, knees, small joints of hands
Responsive to treatment of myopathy
Antisynthetase Syndrome (myopathies)
30% pts: Acute onset of disease Constitutional symptoms Raynauds Mechanics hands Arthritis *Interstitial Lung Disease *Anti-synthetase antibodies (Anti-Jo 1)
amyopathic DM
don’t worry
skin manifest, no musc
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
malignancies with myopathies: adenocarcinomas
cervix lung *ovaries* pancreas, bladder, and stomach (70%) Nasopharyngeal in SE Asians
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)
myopathy/malignancy dx
Muscle enzymes Autoantibodies EMG Tissue biopsy MRI Imaging
musc. enzyme markers myopathies
prognostic?
CK, aldolase
LD, AST/ALT
** treatment should be guided at patient’s strength, not concentration of muscle enzymes
more myopathy dx
ANA (+) in 80%
Anti Ro/La/RNP to rule out underlying CTD
Myositis-specific antibodies (30%)
Ab for mechanic hands, Raynauds, interstitial lung disease
Anti-Jo 1:
Ab against anti-histidyl tRNA synthetase
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
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
myopathy dx: musc. biopsy
Definitive test
Quadricep or deltoid contralateral to muscle found to be abnormal on EMG
leukocytes invading musc.
PM musc. biopsy
inflamm. cells invade healthy musc–>which become rounded and variable in size
DM musc. biopsy
atrophy of fibers near fasc. border
inflamm. cells conc. around BVs (form cuffs) and at border, fiber shrinkage
inclusion body myositis biopsy
musc. fiber w/ vacuoles and inclusion bodies
inflamm. cells btw fibers
skin biopsy myopathies
may be non-sp.
DM skin findings (Gottron’s papules, Shawl sign, erythroderma)
-may avoid musc. biopsy if charac. pres.
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)
these factors do NOT predict disease course/tx response
Gender, race, presence of rash, CK elevation
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
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
goals of myopathy tx
Improve muscle strength
Avoid development of extramuscular complications
Resolution of cutaneous manifestations in DM
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
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
why glucocorticoids fail in myopathy tx
Alternate diagnosis
Glucocorticoid-induced myopathy
Unrecognized malignancy
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
myopathy tx course
No studies addressing optimal duration of therapy
Tapering of Prednisone and Azathioprine/MTX based on clinical improvement, remission, and flares
other myopathy tx consids.
Exercise Aspiration precautions Avoidance of sunlight Osteoporosis prevention Opportunistic infections
refractory myopathy tx
(flares)
Add or modify dose of Prednisone
Add, change, or modify dose of Azathioprine/MTX
recurrent myopathy tx
Rituximab IVIG Cyclosporine/Tacrolimus Mycophenolate Mofetil Cyclophosphamide
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
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
IBM dx
Lab Findings
CK normal/mildly elevated,
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
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
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
spondyloarthropathy presentation
acutely ill, limp synovitis, effusion swollen/tender Achilles tendon (enthesitis) tenderness along plantar fascia elevated synovial fluid WBC elev. ESR, CRP
spondyloarthropathies
Ankylosing spondylitis Psoriatic Arthritis Enteropathic Arthritis (Crohn’s/UC) Reactive Arthritis (formerly Reiter’s Syndrome) Undifferentiated
spondyloarthropathy common features
Inflammation of axial joints (SI joints) Asymmetric oligoarthritis Dactylitis Enthesitis Genital/skin lesions Eye/bowel involvement Association with infectious disorders
spondyloarthropathy genetic marker
dx?
HLA B27 association
not dx, inclusion factor
spondyloarthropathy prevalence
0.5 to 2 percent in Caucasian population
Ankylosing spondylitis and undifferentiated types most common
Reactive arthritis least common
spondyarth. muscskel features
Inflammatory back pain
Most common symptom at onset (70%)
Inflammatory vs. Mechanical back pain
Onset
spondyarth: peripheral arthritis
Acute onset, affects lower extremities (knees,ankles)
Asymmetrical, affecting 1-3 joints
Joint swelling
enthesitis: Achilles tendon
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
dactylitis
“sausage toe/finger”
Differentiated from synovitis by swelling of entire digit
Nonspecific finding
Also seen in TB, syphillis, sarcoid, sickle cell disease
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
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
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
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
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)
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
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
ank. spond dx: chest expansion
limited
Measured at level of 4th intercostal space
arms elevated and hands folded behind head
Change of
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
ank. spond dx misc.
SI joint tenderness
Peripheral joint involvement
Enthesitis/Dactylitis
ank spond responds to…
NSAIDS (bad SEs)
Marked improvement in 24 to 48 hours
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
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
ank spond: extraarticular manifests: bone
Osteopenia/Osteoporosis
Falsely high values on bone density testing due to ankylosing and syndesmophytes
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
ank spond px
Hip involvement Dactylitis Onset 30) Limitation of lumbar spinal motion Poor efficacy of NSAIDS
Male gender, smoking, history of uveitis
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
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
psoriatic arthritis pres.
Distal involvement of DIPs (vs RA-NOT DIPs!)
Asymmetric oligoarthritis
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
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%
“pencil in cup” deformity
seen in psoriatic arthritis
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
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
reactive arthritis triad
postinfectious arthritis, urethritis, conjunctivitis
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
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
react arth clin features
Asymmetric oligoarthritis
Enthesitis
Dactylitis
react arth clin features: extra-art. symps
Dysuria, pelvic pain Diarrhea Conjunctivitis Oral ulcers Skin and genital lesions Nail changes
blennorrhagica
pustular lesions on feet w/ reactive arthritis
circinate balanitis
lesion on penis in reactive arthritis
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%
react arth px
Most have complete resolution 6 months after presentation
Chronic persistent arthritis in small proportion
react arth tx
NSAIDS
Symptomatic relief; do not shorten disease course
Intraarticular steroids
Systemic glucocorticoids
DMARDS-rarely
Sulfasalazine if NSAIDS/steroids not effective
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
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
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
sarcoidosis EMG
peripheral neuropathy, myopathy
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
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
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
sarcoid genetics
HLA (DR17, DRB1*1101) HLA-DQB1*0201 (Lofgren’s Syndrome) BTLN-2 gene on chromosome 6p Negative costimulatory molecule don't worry
sarcoid symps
Nonspecific Fever Sweats Weakness Weight loss Aches and Pains Psychological Issues ORGAN SPECIFIC SYMPTOMS
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
sarcoid testing
CXR
PFTs
CT scan
sarcoid skin
> 1/3 of patients
Classic lesions Erythema nodosum Maculopapular lesions Hyper- and hypopigmentation Keloid formation Subcutaneous nodules
sarcoid: lupus pernio
Bridge of nose
Beneath eyes
Cheeks
Plaque-like induration
Violaceous discoloration
Erode to cartilage and bone
sarcoid: Lofgren’s syndrome
B/L hilar adenopathy
Erythema nodosum
Arthritis (ankles)
Uveitis
sarcoid: eye
Anterior Uveitis 65% Posterior Uveitis 25% Retinitis Pars planitis
Slit lamp
fundoscopic
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
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
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:
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
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
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
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
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
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
sarcoid 2nd line tx
Methotrexate Azathioprine (Imuran) Leflunomide (+/- MTX) Mycophenolate Hydroxycholoroquine
