Medical Manifestations: Endo/Heme/Onc Flashcards
Endocrine diseases w/ rheum manifestations
-DM
-Hyper/Hypo TSH, PTH
-Acromegaly
-Cushings
-Hyperlipoproteinemia
** MSK / Rheum complications in DM **
- Charcot joint, diabetic osteolysis
- Diabetic stiff hands (cheiroarthropathy) - prayer sign
- Amyotrophy, Muscle infarction
- Adhesive capsulitis
- Calcific shoulder periarthritis
- CRPS (shoulder hand syndrome)
- Flexor tenosynovitis (trigger fingers)
- Dupuytren’s contractures
- CTS + Thenar atrophy
- DISH
- Septic joint / OM
** Diabetic cheiroarthropathy pathogenesis**
- Excessive glycosylation of dermal and periarticular collagen
- Decreased collagen degradation
- Increased dermal hydration
** Diabetic cheiroarthropathy risk factors**
-DM duration,
-Glucose control
-Renal/retinal microvascular disease
** Charcot MSK manifestations**
-Midtarsal collapse → Rocker bottom feet
-Painless, swelling, deformity (tarsometatarsal, ankle, knee, hip, spine)
-Bony prominence skin ulcer, infxn
** Charcot radiographs **
5D’s:
- Destruction,
- Debris,
- Density (increased),
- Disorganization,
- Dislocation
** Charcot pathogenesis **
- Sensoral neuropathy + repetitive microtrauma
- Autonomic neuropathy → MORE blood flow
Both of which cause more inflammation, osteoclastic resorption, osteopenia, and #
** Charcot Tx **
- Protected weight bearing,
- Soft casts, good shoes,
- Treat/Prevent skin ulcers
- Neuropathic meds
- Amputation as needed
Diabetic osteolysis
– what is it
– pathogen
Osteoporosis / Resorption of distal metatarsal bones and proximal phalanges +/- acroosteolysis
-Pathogenesis: unknown, ~to charcot
Diabetic amyotrophy manifestations
+what doesnt it have
-Muscle wasting, weakness AND SEVERE pain (proximal muscles: pelvis, thigh, paraspinal, shoulder girdle)
-Distal symmetric neuropathy, autonomic neuropathy
-NO diabetic retinopathy or nephropathy
Diabetic amyotrophy pathogenesis
Injury to peripheral nerves, nerve roots, lumbosacral plexus via:
-Ischemia
-Immune mediated microvasculitis
-Inflammation
Diabetic amyotrophy
– nerve bx findings
– muscle bx
Nerve: Inflammatory infiltrate of blood vessel
-Muscle: fiber atrophy w/o inflammation
Diabetic muscle infarction
– Manifestations
– Ix
Acute onset pain/swelling thigh or calves
-Elevated CPK
-MRI to r/o infxn, malignancy
-Excisional Biopsy
Dequervain’s tenosynovitis pathogenesis
-Microvascular disease affecting nerves & blood vessels
- Protein glycosylation
- ECM protein deposition in skin/ & periarticular structures
Dupuytren’s contracture pathogenesis
Microvascular ischemia → contractile myofibroblasts producing increased collagen
CTS physical exam tests
Tinel’s
-Phalen’s
-Durkan’s
CTS manifestations
Nocturnal paresthesias
-Hand pain
-Pain radiating to elbow/shoulder (Valleix phenomenon)
-Thenar atrophy
CTS pathogenesis
Neuropathy due to:
-Extrinsic compression
-Microvascular disease ischemia of vasa nervorum
SSc DM complication mimickers
Diabetic stiff hand (flexion contracture and indurated digits)
-Distal neuropathy ~ Raynaud’s
-Scleredema diabeticorum (thickened skin upper back/neck)
DM meds causing rheumatic manifestations
OP:
- Glitazone
- SGLT2 (-flozin)
Arthralgia/myalgia:
- DPP4i (-gliptins)
Hypothyroidism MSK manifestations
-Myxedematous arthropathy (noninflammatory; ++viscous 2/2 hyalronic acid)
-Reversible bone marrow edema (~AVN)
-CPPD
TRAP
-Tunnel (CTS)
-Raynauds
-Aching myalgia
-Proximal muscle weakness/stiffness with high CK
HYPERthyroid rheum syndromes
-OP
-Myopathy (painless prox muscle weakness)
-Thyroid acropachy
-Adhesive capsulitis (controversial)
Thyroid acropachy manifestations
Soft tissue hand swelling
-Metacarpal and Phalangeal: clubbing & PERIOSTITIS
Thyroid acropachy associated withwhich thyroid disease
Graves
IIM vs Hypo/HyperThyroid
-CK, Weakness, Biopsy
IIM: CK up, Mild/severe, inflammation
-Hypothyroid: CK up, mild, N
-Hyperthyroid: CK N, mild, N
Thyroid meds causing rheum manifestations
PTU → sysetmic vasculitis or drug induced lupus
-Methimazole → lupus like syndrome
Primary hyperPTH rheum syndromes
-Myopathy w/ NORMAL CK
-Osteoporosis
-CPPD
-Soft tissue calcifications
-Tendon ruptures
-Osteogenic synovitis from subchondral collapse → OA and reactive synovitis
-Osteitis fibrosa cystica
** 5 peripheral radiographic signs of hyperparathyroid or Osteitis fibrosa cystica
2 axial and skull findings**
Peripheral
- Chondrocalcinosis
- Soft tissue calcification
- Osteopenia, osteoporosis
- Brown tumors (lytic lesions in hands)
- Acro-osteolysis
- Subperiosteal resorption and blurred cortical margins
Axial
- Sacroilliitis
- Osteoid deposition at vertebral endplate → sclerotic bands (rugger jersey spine)
- Compression fracture
Skull
- Salt and pepper sign - lucencies caused by trabecular resorption
Knuckle knucle dimple knuckle sign
-What disease
Pseudohypoparathyroidism (PTH resistance) w/ skeleta deformity with a short 4th metacarpal
Acromegaly Rheum manifesetations
MSK:
-OA (knees, shoulders, hips, hands, C/L/S spine), crepitus
-Vertebral fracture
-Prox muscle weakness (normal EMG/CK)
-CTS
-Raynaud’s
-Rare chondrocal
Acromegaly pathogenesis
Excess GH (tumor, central) causes elevation of IGF1 that affect osteocytes, chondrocytes, and fibroblasts
** Acromegaly radiographic findings **
-Soft tissue thickening
-Spade phalanx
-Skull thickening, enlarged sella turcica
-Increased joint/disk space
-Chondrocalcinosis
-Periosteal apposition (diameter thickening) of tubular bones
Excessive GC rheum syndromes
-OP
-AVN
-Prox muscle weakness (normal EMG, CK, biopsy shows t2b muscle fiber atrophy)
-Steroid withdrawal
Steroid withdrawal syndrome manifestations
Aka slocumb’s syndrome
-Myalgias, Arthralgias
-Noninflammatory joint effusion
-Lethargy
-Low grade fever
Familial hyperlipoproteinemia types & associated MSK disorders
Type 1, 4, 5: gout
-Type 2, 3: tendinous xanthomas on digital extensor tendon & achilles tendon → tendinitis; tuberous xanthoma over extensors ~RA nodules or tophi
-Type 2: migratory inflamm arthritis ~rheumatic fever (no cholesterol crystals)
-Type 3: osseous xanthomas in long bones → #
Bloody synovial fluid: hemarthrosis vs traumatic tap
-How to use Hct
Hct ~ peripheral blood = Traumatic
-Hct < peripheral blood = Hemarthrosis
** Hemarthrosis causes**
-Vascular: ateriovenous fistula, ruptured aneurysm
-Heme: Hemophilia, VwD, Sickle Cell, Thrombocytopenia, myeloproliferative dz , hemoglobinopathy
-Infxn: lyme, septic arthritis
-Trauma: injury, fracture, post-surgical
-Autoimmune: Ehlers Danlos syndrome, Pseudoxanthoma elasticum,
-Metabolic: Scurvy, Charcot joint, Post dialysis, Gaucher’s disease
-Meds: Excessive anticoagulation or thrombolytics
-Inflammatory: Crystal disease (hydroxyapatite), Amyloid
-Neoplasm: pigmented villonodular synovitis, tumor mets to joints, 2ndary tumor of synovium, hemangioma
Synovial fluid findings in fracture
- Fat globulesor cholesterol emboli
- Direct visualization or
- Oil red O staining
INR cutoff for joint aspiration
4.5
Hemarthrosis Tx
-Therapeutic Tap+/- IA GC
-Ice, compression
-Analgesia
-Reversal of supratherapeutic INR
Hemophilia Rheum problems
Acute hemarthrosis
-Subacute → Chronic → end stage arthropathy
-Intramusculaar or soft tissue hemorrahge
-Subperiosteal hemorrhage → Pseudotumor
-Septic arthritis
Hemarthrosis Tx in Hemophilia
Replace deficient factor to >30% (2% increase per unit/kg body weight)
-Rest, Ice, Analgesia, Compression
-IA GC
-PTto prevent muscle atrophy and contracture
Risk factors for septic arthritis coexisting w/ hemarthrosis
-Focus for seeding: Arthroplasty, joint damage
-Portal of entry: previous arthrocentesis, IVDU
-Infection RF: Fever, leukocytosis, HIV
-Persistent activity/pain despite factor replacement
Hemophilia acute radiographic findings
Soft tissue swelling,
-Effusion,
-Increased synovial density (iron deposition)
Hemophilia chronic radiographic findings
-Erosions
-OA (cysts, osteophytes and sclerosis)
-Wide intercondylar notch (femur/humerus)
-Inferior patella flattening
-Talar flattening
Chronic hemophilic arthropathy Tx
Prevention:
- Prophylactic factor 8 infusion (factor goal >5%)
-Nonweightbearing periods for synovitis to regress
-PT for joint stability and muscle strength
-IA GC
-NSAIDs (Cox2 specifici eg celecoxib)
-Chemical synoviorthesis with rifampicin
-Synovectomy (arthroscopic if chronic synovitis or radioisotope if factor inhibitor)
-THA
Sickle Cell disease Rheum manifesetations
-Muscle infarct and focal necrosis, rhabdomyolysis
-Bone infarct (dactylitis, AVN) - can cause transudative noninflamm effusion
-Hemarthrosis
-Chronic synovitis
-Hyperuricemia and gout
-Septic arthritis, OM (Salmonella, S Aureus)
RF infection in sickle cell patient
-Functional asplenia (decreased bacterial clearance)
-Neutrophil dysfunction at lower O2 tension
-Decreased opsonization and IFNg production
SCD spine XR findings and pathogenesis
-Codfish vertebrae (central cup like indentation) from osteoporotic vertebrae weakness from marrow expansion
AVN femoral head Tx
Nonweightbearing to prevent collapse and allow revascularization
-Ortho consult: core decompression of femoral head
-Joint replacement
Hydroxyurea rheumatic mimics
-Hand foot syndrome (Palmar plantar erythrodysethesia +/- blisters) mimics systemic vasculitis
-Alopecia
-DM like or SSc like sydromes
-DM like rash
MDS autoimmune syndromes
- Vasculitis: PAN, LCV, GCA, uveitis, GN, pleuropericarditis
-CTD: RP, SLE, Myositis
-Inflamm polyarthritis: PMR,
RA, Sjogrens, R3SPE - Positive antibodies (ANA, ANCA, RF, APLA, Cryo)
-Neutrophilic disease: Sweet’s, pyoderma gangrenosum
-Other: AIHA
MDS autoimmune syndrome pathogenesis
Accelerated apoptosis of maturing hematopoeitc cells increasing cytokine production (IL6, TNFa, IFNg)
MDS autoimmune syndrome tx
GC
-DMARD (HCQ, MTX)
-Biologics
Mechanisms of malignancy causing MSK sx
-Tumor invasion
-Paraneoplastic
-Treatment related
-Malignancy 2/2 dz activity or immunosuppression
MC cancer causing bone mets
KTL PB
-Kidney
-Thyroid
-Lung
-Prostate
-Breast
MSK features of leukemia and lymphoma
Polyarthralgia/arthritis
-Bone pain (subperiosteal infiltration)
-Hemarthrosis
-Gout from chemothearpy
-Septic arthritis
XR findings leukemia/lymphoma
- Metaphyseal radiolucent band
- Osteolytic lesions
Paraneoplastic syndromes assoc’d w/ malignancy
Myopathy
-Arthropathy
-Vascular
-Cutaneous
-Miscellaneous
Paraneoplastic myopathies assoc’d w/ malignancy:
DM, PM, Lambert Eaton
Paraneoplastic arthropathies assoc’d w/ malignancy:
-HPOA,
-Amyloid,
-2ndary gout,
-Carcinomatous polyarthritis,
-Atypical PMR
-RS3PE
Paraneoplastic vascular manifestations assoc’d w/ malignancy:
-PAN,
-LCV, HSP,Cryo
-Granulomatous vasculitis,
-Digital necrosis,
-Strokes
DERM manifestations assoc’d w/ malignancy
-Sweet’s,
-Palmar fasciitis,
-Scleroderma,
-Paniculitis,
-Erythromelalgia,
-Multicentric reticulocytosis
Paraneoplastic miscellaneous manifestations assoc’d w/ malignancy
Septic arthritis,
SLE-like syndrome APLA,
Oncogenic osteomalacia,
Sarcoid,
Lymphomatoid granulomatosis
Atypical PMR features
-Poor steroid response
-Asymmetric
-ESR normal or VERY high
-Adenopathy
RF Cancer in DM/PM
Older age DM/PM onset
-Treatment resistant myositis
-Pharyngeal / Diaphragmatic involvement
-LCV
-Amyopathic DM
-DM with cutaneous necrosis
-TIF1 or NXP2
**HPOA syndrome **
Clubbing fingers/toes
-Periostitis of tubular bones
-Arthritis (noninflamm synovial fluid)
-
-Localized form: hemiplegia, aneurysm, infective arteritis, patent ductus arteriosus
HPOA causes
Primary (genetic)
-Cancer (GI, hepatic)
-Chronic infections (lung, subacute IE, HIV)
-Cystic fibrosis
-Congenital cyanotic heart disease
-IBD
-Cirrhosis
-Graves
-Voriconazole
HPOA pathogenesis
Hypoxemia increases circulating growth factors, platelet-derived growth factor (PDGF), VEGF, PGE2 causing:
- Smooth muscle proliferation, - Angiogenesis
- Collagen deposition.
- Effect on osteoblasts and osteoclasts
HPOA Tx
Zoledronic acid
Carcinomatous polyarthritis features
- Explosive onset
– Asymmetric oligo/poly
– Late age of onset
– Lower extremity predominant
– Spares wrist and small hand joints
– NO erosions, nodules, RF
– NORMAL XR
Sweet’s syndrome features
–Abrupt onset raised/painful papules/plaques of face, neck, trunk, dorsum of hands
– Fever
– Peripheral neutrophylic leukocytosis
– Dense dermal neutrophilic infiltrates w/o vasculitis on biopsy
Sweet syndrome causes
-Infxn: GI/GU, HIV, TB, Viral Hep, Chamydia
-Cancer: Heme> solid
-Autoimmune: IBD, SLE, RA, RP, Sarcoid, Thyroid
-Autoinflmmatory: VEXAS
-Primary immunodeficiency
-Pregnancy
-Drugs: GCSF, antibiotics, AEDs, NSAIDs, Imuran, Diclofenac, hydralazine, antiHIV, clozapine, PTU, Lasix,
Ovarian carcinoma rheum syndromes
-SLE -like syndrome, +ANA
-DM/PM
-Sweet’s
-Palmar fasciitis
-Adhesive capsulitis
-CTS
-Fibro, CRPS
Palmar fasciitis syndrome features
-Painful symmetric digital contractures
-Palmar fascia fibrosis (woody)
-CRPS
-Polyarthritis
Palmar fasciitis histology
-Fibrosis with increased fibroblast and mononuclear cells
**-NO collagen deposition
**
-IgG deposit in palmar fascia
Palmar fasciitis Tx
Treat underlying tumor
-Poor response to chemo, NSAID, GC
Shoulder hand syndrome sx
Shoulder pain with decreased ROM
-Hand puffiness and stiffness with vasomotor instability
CTD’s assoc’d with cancer (and risk factors)
-RA (dz duration/activity, Felty’s, immunosuppresion)
-Sjogren (extraglandular: pupura, ulcers, adenopathy, splenomegaly, cryo)
-SLE (adenopathy, splenomegaly)
-Discoid SLE (plaques >20y)
-DM/PM (older, skin ulcers, NXP2)
-SSc (RNApol3, pulm fibrosis, barett’s metaplasia)
-Eosinophilic fasciitis (thrombocytopenia, aplastic anemia)
-AAV (cyclo)
-Paget’s
Nonbiologic DMARDs (and assoc’d cancer)
MTX (lymphoma)
-Aza (NHL)
-Cyclosporine (lymphoma, skin)
-MMF (CNS lymphoma, skin)
-Cyclo (lymphoma, bladder ca)
What rheum manifestations
- Bleomycin
- Gemcitabine
Bleomycin: Raynaud’s, SSc
Gemcitabine: SSc like illness w/ critical digital ischemia
Taxanes → what rheum manifestations
Arthralgias
-Myalgias
-Subacute cutaneous lupus
Interferon alpha → what rheum manifestations
Arthralgias
-SLE like syndrome
-Positive autoantibodies
-Autoimmune thyroid dz
Immunotherapy mech of rheum symptoms
-ICI block CTLA4, PD1 PDL1,
(negative signals on activated T cells) = increased autoreactive T cells and inflammatory cytokines (TNFa, IL-6/17)
5 grades of ICIs
1= asymptomatic or mild
-2= limit iADL
-3= limit ADL
-4 = life threatening
-5 = death
ICI Tx
Steroids
-DMARDs: MTX, HCQ, SSZ
-Anti TNF
-Anti-IL6
IRAE manifestations
PsA
-Vasculitis
-PMR
-Sjogrens
-Myositis
-GN
-Sarcoid
-Uveitis / Scleritis / Retinitis
-Optic neuritis
-DRESS
-Myocarditis / Pericarditis
-Transverse myelitis
Rheum cause of Corneal melt**
RA (most common)
Vasculitis
ANCA vasculitis (GPA most common)
PAN
Relapsing polychondritis
SLE
Sjogren’s
SSc (rare)
GCA (very rare)
IBD
Sarcoidosis
Rheum cause of lens dislocation**
Marfan
Rheum cause of scleritis**
RA
GPA
RPC
SLE, UCTD
IBD, Seroneg, Poderma gangrenosum
Sarcoid
Behcet, Cogan
TAK, GCA, PMR
HUVS, Hep C related vasculiti
Rheum cause of sterile conjunctivitis**
-SS,
-KD,
-ReA
-Vasculitis
- RA
- SLE
Rheum cause of anterior uveitis**
-Seronegative (PEAR)
-Behcet
-Sarcoid
-KD
-JIA, Kawasaki, TINU (tubulointerstitial nephritis & uveitis)
- HUVUS
- Cryo
Rheum and nonrheum cause of intermediate uveitis**
Behcet, Sarcoid, Pars Planitis (snowbanks/ snowballs)
Non rheum: Multiple sclerosis, TB, HSV, Whipples,
Rheum and nonrheum cause of posterior uveitis**
Behcet, Sarcoid, SpA (PsA, IBD), SLE, VKHS (vogt koyanagi harada syndrome)
Nonrheum: toxo, birdshot retinochoroidopathy, cancer related, histoplasmosis, syphilis, HSV, VZV, CMV
Rheum and nonrheum cause of panuveitis**
Behcet, Sarcoid, Blau syndrome, IBD
Toxo, VKH, TB, HSV, Fungal
Rheum cause of retinal vasculitis**
Behcet,
Sarcoid
SLE
APS
RA
AAV, GCA, Susac
Nonrheum: CNS lymphoma, leukemia, toxo, TB, lyme, syphilis, HSV, VZV, cat scratch, CMV, HIV, Whipples, Pars planitis, birdshot
Rheum cause of orbital inflammation**
IgG4,
Sarcoid,
Behcet
GPA,
RA
Thyroid assoc’d
** Peripheral nervous system manifestations in CTD**
-Length dependent sensory polyneuropathy
-Mononeuritis multiplex
-GBS/CIDP
-Distal axonal polyneuropathy
-Compression neuropathy
Anti-Gu disease association
GAVE
-SSc
-SLE
-UCTD
True/ False
– FMF + AA Amyloid → erosive arthritis
– AL Myloid + MM → HF
– Ank spond + AA amyloid → Proteinuria
– Dialysis pt w/ B2 microglobulin amyloid → periarthritis, CTS, effusive arthropathy, erosive bone lesion
-F
-T
-T
-T
Which CTD with which ILD?
-UIP
UIP = RA >PM/DM, SSc, SS, SLE
Which CTD with which ILD?
-LIP
SJ > RA
Which CTD with which ILD?
-NSIP
SSc, DM/PM > SJ, MCTD, SLE, RA
Which CTD with which ILD?
-BOOP/ COP
DM/PM, RA, SJ, SLE
Which CTD with which ILD?
-AIP
AIP = SLE, DM/PM
** What is a scleroderma mimic associated with diabetes?**
- Diabetic Cheiroarthropathy
- Scleredema diabeticorum
** Longstanding diabetic comes with hand weakness and paresthesia:
-Name 3 possible diagnoses?
-Name 2 investigations?**
Three diagnoses:
1. Carpal tunnel syndrome
2. Diabetic neuropathy (length dependent, so must already have symptoms in their feet)
3. Radiculopathy (if unilateral)
4. CRPS
Two investigations:
1. NCS/EMG
2. Cspine imaging, if concerned for radiculopathy
** Charcot disease associated processes **
Anything that causes peripheral neuropathy
- DM
- EtOH neuropathy
- Infection: Neurosyphylis, Leprosy
- Trauma: Spinal cord injury
- Cerebral palsy
** Physical exam maneuver will distinguish between neuropathic joint vs osteomyelitis **
- “Dependent Rubor test” - elevating foot REDUCES erythema in Charcot but NOT in infection
- Probe to bone points to infection > Charcot
- Ulcers >2cm more specific for osteomyelitis
- Sausage Toe seen in OM
- OM typically affects toes/forefoot (vs midfoot in Charcot)
** Imaging technique other than xray/CT/MRI to help make diagnosis between Charcot vs OM**
- WBC scan – negative for Charcot, positive for OM.
- Bone scan – show uptake in both OM and Charcot.
- MRI – features of OM and Charcot overlap on MRI. Sens 90%, Sepc 79%.
DDX symmetric small joint arthropathy and liver dz
Hemochromatosis
Amyloid
Celiac
RA w/ NASH
Hep B/C
PBC, PSC
Hemochromatosis: features of the arthropathy, findings on xray
- Symmetric: hands, wrists, knees, hips
- Lack of warmth/effusion unless CPPD
- Chondrocalcinosis
- Hook osteophytes on MCP2/3 >4/5
- OA: subchondral sclerosis/cysts, flattened metacarpal heads, uniform joint space loss
- OP
- Wrist involvement: degeneration between carpal bones and CMC joints, not at radiocarpal joint (as in CPPD)
Hemochromatosis: extraarticular manifestations
- Pituitary - infiltration causes hypogonadism
- Skin: melanin deposition = bronzing
- Porphyria cutanea tarda,
- Heart: Cardiomyopathy, CHF
- Liver: cirrhosis, hepatomegaly, elevated enzymes
- Pancreas: Diabetes
Hemochromatosis: workup
CBC, PTH, Ca, PO4, Mg, renal function
Iron studies
Liver enzymes
HFE C282Y if fam hx
MRI/liver biopsy only if iron overload or diagnostic clarity
** What is pigmented villonodular synovitis PVNS**
- Benign neoplasm causing hypervascular and proliferative synovial/tendon sheath and erosive monoarthritis
- MC: hands, wrists, knee, hip, ankle
- Tx = synovectomy or radiation if refractory
** List 5 dermatological lesions that are associated with arthropathy. Outline the features of 1 of these **
LCV: RA, SLE, AAV, etc
E nodosum: Behcet, IBD, sarcoid
Pyoderma: RA, Behcet, SLE
Lupus pernio: SLE
Cutaneous SLE: SLE
Psoriasis: PsA
Keratoderma blenorrhagicum: ReA
Dermatitis herpetiformis: Celiac
List 5 common causes of Anterior Ischemic Optic Neuropathy (AION)
Arteritic AION
- GCA
Non-arteritic AION (sudden, painless vision loss/blurring in one eye, usually upon waking 2/2 lower BP during sleep)
- Anything reducing perfusion or increasing bloodflow resistance to optic nerve
- HTN
- OSA
- Smoking
- Hypercholesterolemia
- Anemia
- Chronic renal failure
- Migraine
** CTS DDX**
PRAGMATIC.
Pregnancy (20%)
RA (any inflammatory arthritis) Acromegaly
Glucose (diabetes)
Mechanical (overuse, occupational) Amyloid
Thyroid (myxedema)
Infection (tuberculosis, fungal) Crystals (gout, pseudogout)
** What are the complications of uveitis?**
Blindness/vision loss
Cataracts
Glaucoma
Choroidal neovascularization
Cystoid macular edema
Optic neuropathy
Band Keratopathy (calcium deposition in the corneal epithelium)
Posterior synechiae (adhesion of the iris to the lens which lies posterior to it)
** Name three infectious causes of uveitis**
HSV, CMV, EBV , VZV
Lyme
TB
Bartonella
Cat scratch
** List 3 clinical or laboratory features of uveitis seen in JIA, ankylosing spondylitis, psoriasis, and sarcoidosis **
JIA: asymptomatic uveitis, young female, ANA+
Ank spond: HLAB27+, recurrent course, sudden onset, unilateral but can alternate
PsA: HLAB27+, can be bilateral posterior, or anterior
Sarcoid: can be anterior, intermed, posterior, or panuveitis.
**What is synechiae? **
Adhesions that are formed between adjacent structures within the eye usually as a result of inflammation
Affects aqueous movement causing pressure buildup posteriorly and angle closure glaucoma
Basic exam elements for red eye
Visual acuity
External structure evaluation: swelling, blepharitis, en/extropion, proptosis, lacrimal gland enlargement
palpebral/bulbar conjunctiva
Ciliary flush (in uveitis, keratitis, acute angle closure glaucoma)
Pupillary size/shape/light response
Fundoscopy for retinal ischemia or vessel abnormality
Episcleritis vs scleritis
Episcleritis - erythema/discomfort WITHOUT significant pain (good prognosis)
- Brighter red that resolves w/ phenylephrine
Scleritis - severe persistent pain, erythema, photophobia, tearing, decreased visual acuity
-Can hv blue/purple hue
-Avascular areas can suggest scleromalacia perforans
Scleritis subtypes
Diffuse - most benign;
Nodular - tender nodule (dark red)
-Necrotizing - destructive l eg scleromalacia perforans (scleral necrosis/thinning WITHOUT pain/redness)
-Posterior - no redness (hard to dx), and minimal visual change or pain
Episcleritis autoimmune diseases
RA
IBD
Vasculitis
SLE
Other
Scleritis autoimmune diseases
RA
GPA
RPC
SLE, UCTD
IBD, Seroneg, Pyoderma gangrenosum
Sarcoid
Behcet, Cogan
TAK, GCA, PMR
HUVS, Hep C
Nonrheum cause episcleritis or scleritis
HSV
Aspergillus
TB
Lyme
Syphilis
Pseudomonas
Bisphophonates
Trauma
Cancer
Episcleritis tx
Cold compress and lube
Topical or PO NSAIDs
Topical or PO GCs
Scleritis Tx
PO NSAIDs - for nonnecrotizing anterior
GC if refractory
MTX, AZA, MMF, CNI, CYC , TNF, Ritux
Diff between Anterior vs Intermed vs Posterior uveitis
Pain and photophobia in anterior, not in others
Others have blurred vision, floaters,
Causes that present with any form of uveitis
Behcet
Sarcoid
Lyme
Syphilis
HSV
Uveitis presentation in Seroneg
Ank Spond and ReA = SUDDEN onset, unilateral
PsA and IBD = insidious bilateral chronic
Uveitis presentation JIA
ASYMPTOMATIC, NORMAL appearing eye
Uveitis rheum causes and their onset / laterality
HLAB27
PsA
JIA
Sarcoid
Behcet
HLAB27: SUDDEN, UNIlateral
PsA: insidious, BILATERAL
JIA: insidious, BILATERAL
Sarcoid: SUDDEN, BILATERAL
Behcet: SUDDEN, BILATERAL
Uveitis Tx
Anterior: topical GC (PO if needed), mydriatic/cycloplegic agents if pain
Uveitis in any segment: periocular/ intravitreal /PO GC, MTX, AZA, MMF, LFN, CNI, CYC, TNF (NOT etanercept), Ritux
Retinal vasculitis presentation
Bilateral
Painless blurred vision,
Scotomata,
Floaters
Difficulty perceiving colors
Straight lines appear wavy
Retinal vasculitis Tx
GC for all (even infectious causes + abx)
Aza, CYC, MMF, TNF, Ritux
Susac features
Triad:
Encephalopathy (involves corpus callosum)
Branch retinal artery occlusion
Sensorineural hearing loss
Irreversible vision/hearing loss
Susac Tx
Pulse steroids then 1mg/kg for 2-4wks
+/- IVIG, PLEX
MMF, CYC, AZA, CNI, Ritux
Orbital inflamm disease rheum causes
GPA
Sarcoid
IgG4RD
RA, Sjogren’s
IBD
Behcet
SLE,
AOSD
Amyloid
Histiocytic disorders
Nonrheum: thyroid, cancer, idiopathic
Orbital inflamm disease presentation
Pain
Orbital swelling
Diplplia
Chemosis
Proptosis
Orbital inflamm disease tx
Treat underlying
Immune mediated inner ear disease (IMIED) presentation vs Idiopathic sudden SNHL vs Menieres
IMIED: Rapidly progressive BILATERAL irreversible SNHL with vestibular sx (vertigo, tinnitus, aural fullness)
Idiopathic: unilateral and NOT assoc’d w/ vestibular symptoms. Fails Weberm and humming localizes to good ear
Meniere’s happens over years (vs weeks/months in IMIED)
IMIED Tx
GC 1mg/kg x2-4wks
Quick taper if no response, slower taper over 2-3mo if response at repeat hearing test
Cochlear implants
Conditions can cause acro-osteolysis?
SSc
PsA
JIA
Sarcoid
HyperPTH
