MS Flashcards
Amyloidosis H and P ?
Periorbital purpura
Fatigue Involuntary weight loss Macroglossia (uncommon) Kidney and liver edema CHF crackles JVD Carpal tunnel syndrome - cause of the swelling
Amyloidosis screening ?
Serum or urine Immunohistochemical analysis
AL– detectable immunoglobulin light-chain protein Not diagnostic ( but it gives you a clue)
AA– detect precursor protein from which AA amyloid fibrils derive
AA type then we can detect the protein
Amyloidosis Disgnosis lab ?
Congo red stain - apple-green birefringence when viewed under polarized light (cotton-wool dye)
Tissue biopsy
Bone marrow biopsy - AL– monoclonal population of plasma cells
Amyloidosis Tx: AL ?
Corticosteroids
Melphalan
Autologous stem cell transplant
Amyloidosis Tx: AA ?
Corticosteroids
TNFs - biologics
humera etc.
Chlorambucil
Autologous stem cell transplant
high tx ass. mortality
Behcet’s syndrome
Recurrent, painful aphthous ulcers
mouth and genitals
Lesions
tender, erythematous, papular
resemble erythema nodosum, but will ulcerate
EN does not ulcerate
Pathergy phenomenon– formation of a sterile pustule at the site of a needle stick
couple days later it is going to result in a sterile pustule
Uveitis (anterior or posterior)
Nonerosive arthritis
Neurologic
cranial nerve palsies, seizures, encephalitis, mental disturbances, and spinal cord lesions
sxs. if neurologic involvement
Behcets tx ?
Corticosteroids
Topical -for oral aphthous ulcers
Systemic -for vision-threatening uveitis
Biologics
Mainstay, with steroids
Colchicine
0.6 mg once to three times daily orally
Corticosteroids
1 mg/kg/d of oral prednisone
are a mainstay of initial therapy for severe disease manifestations
Infliximab or cyclophosphamide is indicated for severe ocular and central nervous system complications
Behcet’s syndrome patho ?
Systemic perivasculitis
all through the body there is inflammation of blood vessels
Endothelial swelling
Polymyalgia rheumatica H and P ?
Proximal joint pain and stiffness Neck, shoulder, and pelvic Bilateral and symmetrical osteoarthritis is not always symmetrical ( RA is?) Worst in the morning and after rest
Fatigue
Fever
Weight loss
Depression
jaw claudication - hurts to chew and causes pain in the master muscle with chewing - sxs. of GCA
Polymyalgia rheumatica labs ?
ESR is markedly elevated ( +40 mm/ hr)
not really helpful
Temporal arteritis is confirmed by biopsy
GCA - painful temple - worry about blindness
Polymyalgia rheumatica tx ?
Low dose corticosteroids 20 mg/day
If temporal arteritis ( then bump up dose cause we need to get the inflammation down)
High dose corticosteroids 40-60 mg/day
Polymyositis H and P ?
Symmetric proximal muscle weakness
insidious, painless
muscle atrophy - more concavity to the fossa
Dysphagia
Polyarthralgias
Dermatomyositis
Gottron’s papules
Rash (malar or heliotrope)
Polymyositis labs ?
Step 1
CK
Aldolase - blood test to check for algalsae levels and it is found it high amount in muscle tissue
ANA - anti nuclear ABS - this is for LUPUS
Step 2 Antibody testing anti-Jo-1 antibody anti-Mi-2 anti-SRP anti-155/140
Step 3
Muscle biopsy
Polymyositis Tx ?
High-dose steroids 1-2 mg/kg/day (this is a lot of prednisone)
control the inflammation
Methotrexate - not a lot of studies proving it work
Consider other DMARDs
Muscle strengthening
Pain control
Rheumatoid arthritis H and P ?
Arthralgia, fatigue, fever
Joint destruction
Bilateral
MCP and PIP joints
MTP and PIP joints
Valgus deformity
Swan neck deformity
Boutonniere deformity
Rheumatoid nodules - along the extensor surface of the arm - firm, 1 cm big and contender and they occur at pressure points ( may go away with tx)
Rheumatoid arthritis labs ?
ESR– elevated
CRP– Elevated
CBC – anemia of chronic disease
1 point
Rheumatoid factor
Nonspecific
Positive 80%
even if they gave RA doesn’t mean they will have this factor
Anti-cyclic citrullinated peptide (ACPA) antibodies
Nonspecific
Positive 95%, better test for RA
can be positive years before sxs.
XR joints Soft- tissue swelling - cause synovial proliferation Juxtarticular erosions Joint space narrowing bone out of alignment
Synovial fluid– to exclude gout and septic arthritis
3,000-50,000 WBC
Rheumatoid arthritis Tx ?
1. Synthetic DMARDs
Methotrexate
Hydroxychloroquine
Sulfasalazine
Leflunomide
Minocycline
- Biologic DMARDs
Injection or IV infusion ( once a week or once every 2 weeks)
these are really expensive - Low-dose corticosteroids
possible adverse reactions
If severe joint destruction
Reconstructive/replacement surgery
Rheumatoid arthritis addition H and P ?
Morning stiffness may last for hours ( osteoarthritis it only takes a coupe min to get loosened up)
Stiffness better with activity, worse after rest
Cervical spine may be affected, but rarely involves thoracic or lumbar spine
Synovial proliferation (pannus) around the wrist can compress median nerve and cause carpal tunnel syndrome
Juvenile Idiopathic Arthritis H and P: Systemic ( Still disease) ?
Systemic ( Still disease)
Spiking fevers ( 39° to 40° C; 102.2° to 104° F) - rabbit ear pattern of fever spiking coinciding with this rash below
Salmon- pink maculopapular rash
appearing in the evening and with the fever
Myalgias
Polyarthralgias
Minimal articular findings
Hepatosplenomegaly
Pericardial effusion
Juvenile Idiopathic Arthritis Tx ?
Stop joint inflammation, restore normal function
referrals to PT/ OT to get them moving
NSAIDs and glucocorticoids (PO or intraarticular) are mainstay
DMARD - Methotrexate first, then biologics
Refer to pediatric rheumatologist - cause they need to be monitored very closely
Juvenile Idiopathic Arthritis types ?
Systemic (Still disease) (10-20%)
Oligoarticular (40%) - few
Four or fewer joints
Polyarticular (35%)
Five or more joints
Psoriatic (<10%)
Enthesitis-Related (<10%)
Undifferentiated
Juvenile Idiopathic Arthritis H and P: Polyarticular - Seropositive ?
Resembles adult RA
symmetric involvement in the hands and feet first progressing symmetrically along the body
Symmetric involvement, often hands and feet
Low- grade fever
Fatigue
Rheumatoid nodules - not in everyone
More often an aggressive form in kids then in adults
Juvenile Idiopathic Arthritis H and P: Oligoarticular ?
Morning stiffness
Asymmetric pattern
Knees common, large and swollen - not as painful as what it looks like it should be
Risk of iritis, asymptomatic – can lead to blindness
Juvenile Idiopathic Arthritis H and P: Polyarticular - Seronegative ?
Younger children
Girls > boys
Large joints – knees, ankles, wrists
Can have active arthritis for years without erosive changes - this is a good sign , it is not causing damage for a long long time and sometimes these kids can convert to seropositive
Can convert to seropositive
Juvenile Idiopathic Arthritis H and P: Psoriatic Arthritis ?
Psoriatic rash or family hx of psoriasis
Arthritis, sacroiliitis, sausage digits ( swollen or puffy), nail pitting (yellow on nails like psoriasis)
Anterior uveitis
Variable prognosis
Remissions and exacerbations
Juvenile Idiopathic Arthritis H and P: Enthesitis - Related ?
Inflammation at the site of attachment of tendon/ligament to bone
INFLAMMATION AT THE SITE NOT AT THE BONE
Pain even without true arthritis
Mainly lower extremities
Mainly boys 8-12 yo
+/- associated with IBD
BLOOD diarrhea
Juvenile Idiopathic Arthritis: Chronic synovitis ?
(+6 weeks)
inflammation of the synoviall lining - decrease ROM, swelling, joint is stiff
Juvenile Idiopathic Arthritis dx labs ?
No specific diagnostic tests
CBC
Rheumatoid factor
ACCP antibody
ESR– normal or elevated
CRP– normal or elevated
ANA– increased often for oligoarticular
Higher likelihood of uveitis
Radiographs
Bone scan
Systemic lupus erythematosus drugs that may induce ?
Procainamide Hydralazine Isoniazid Methyldopa Quinidine Chlorpromazine
Systemic lupus erythematosus H and P ?
Often relapsing and remitting pattern
Fatigue, fever
Malar rash - no scaring
Discoid rash - tends to scar - usually in circles
Photosensitivity
Arthralgias
Oral ulcers (painless)
Lymphadenopathy
Renal symptoms
polyuria, nocturia, foamy urine
Systemic lupus erythematosus dx labs ?
CBC
Anemia
Leukopenia
Thrombocytopenia
BUN– may be elevated
Creatinine– may be elevated
these two only if renal involvement
Urinalysis– possible casts, proteinuria
ESR– elevated usually
ANA– positive 99%, nonspecific
Serum complement ( C3 or C4)– low, can monitor for progression
Antibodies to Smith antigen– elevated, can monitor for progression
Anti-double-stranded DNA– elevated, can monitor for progression
Antihistone antibodies– positive in drug-induced lupus
Systemic lupus erythematosus
Patient Education
Exercise
Sun protection
Pharmacology
NSAIDs - for joint pain
often used for musculoskeletal complaints
Antimalarials ( hydroxychloroquine or quinacrine)
may be used for musculoskeletal complaints and cutaneous manifestations
Corticosteroids (as needed for flares)
Topical or intralesional preparations are often used for cutaneous manifestations
Low- or high- dose oral corticosteroids are used for disease flares and tapered as symptoms resolve.
Methotrexate
used at low doses for arthritis, rashes, serositis, and constitutional symptoms.
Scleroderma H and P ?
Skin
Swelling and tightness in the fingers (sclerodactyly) and hands. May spread to trunk and the face
Raynaud phenomenon
vasospasm of the digital arteries
seen in more than 75% of patients
Musculoskeletal pain
GERD
Limited scleroderma (CREST syndrome)
“Skin Score”
Rate thickness of skin 0-3
17 locations
Helps to monitor progression
High degree of subjectivity
Scleroderma labs ?
ANA– 90% of patients with diffuse type, but nonspecific
Anticentromere antibody—positive with CREST (limited type)
Anti-SCL-70– may be positive with diffuse disease
Indicates poor prognosis
Scleroderma tx ?
No cure
If reflux
PPI
If renal disease
ACEI
If Raynaud
Calcium channel blockers
If pulmonary hypertension
Immunosuppressants
Early and aggressive tx needed
Scleroderma H and P: diffuse ?
Peripheral circulation
Obliterative vasculopathy
Lungs
Fibrosis, Pulm HTN
GI
Constip, GERD, “food stuck”
Renal
Less often, use ACEIs
Cardiac
Ischemia, fibrosis
MSK
Mild to erosive arthritis, joint contractures
Sicca Complex
Dry eyes and mouth
Sjogren syndrome H and P ?
Sicca features
Dry mouth (xerostomia)
food can stick to the mucosa
tooth decay cause no saline
oral infections
Dry eyes (xerophthalmia or keratoconjunctivitis sicca)
more potential for infection
itchy griddy eyes
Parotid glands may be enlarged
Sjogren syndromen labs ?
Initial RF ANA anti- Ro antibodies anti- La antibodies
More diagnostic
Schirmer test - evaluates tear secretions by the lacrimal glands
filter paper placed in the lower eyelid for 5 minutes
Less than 5 mm wetting is positive for decreased secretions
Biopsy of the lower lip mucosa
RF positive in 40 -50% - half the time
ANA positive in 80% - a lot of the time
anti- Ro antibodies positive in 60%
anti- La antibodies positive in 40%
Sjogren syndrome tx ?
Patient Education
Management is mainly symptomatic, with the goal of keeping mucosal surfaces moist
This can be achieved by using artificial tears and saliva, increased oral fluid intake, and ocular and vaginal lubricants
Pharmacology
- Pilocarpine may increase saliva flow
- Cyclosporine drops may improve ocular symptoms
Polyarteritis nodosa H and P ?
Skin lesions
palpable purpura
livedo reticularis
lacy kinda skin
Fever/chills
Anorexia
Weight loss
Abdominal pain- “intestinal
angina”
Peripheral neuropathy
Arthralgias/Arthritis / Myalgias
Hypertension, CP, CHF, MI
Edema
Oliguria
distal ishemica that can lead to gangrene
Livedo reticularis is thought to be due to spasms of the blood vessels or an abnormality of the circulation near the skin surface. It makes the skin, usually on the legs, look mottled and purplish, sort of a net-like pattern with distinct borders
Polyarteritis nodosa labs ?
Step 1
ESR– elevated
CRP– elevated
UA– may have proteinuria, hematuria ( if kidney involvement )
Step 2
ANCA– suggestive, not diagnostic
HBsAg– Hepatitis B may be causative
Diagnosis
Vessel biopsy - might show you necrotizing arteritis
Angiography
Nerve conduction studies
Polyarteritis nodosa tx ?
High doses of corticosteroids
Maybe methotrexate or azathioprine
Consider
- Cytotoxic drugs (cyclophosphamide)
Immunotherapy
Osteomalacia (Rickets) History ?
Delayed growth
Pain in the spine, pelvis and legs
Muscle weakness
