Darrow Lectures Flashcards
criteria for SLE? TQ
B3O1R1N1 with D3ermA1titiS1
SOAP BRAIN MD
S- Serositis (pleuritis or pericarditis) - affects lungs and heart
O- Oral Ulcers (vagina, nose, alopecia)
A- Arthritis - with or without active synovitis. - Can get reversible swan neck deformities.
P- Photosensitivity
B - Blood Disorder (anemia, leukopenia, thrombocytopenia*)
R- Renal Disorder (proteinuria or cellular casts)
A- ANA
I - Immunologic Disorder (ds-DNA, or Sm, or APA, or FPSTS)
N- Neurologic Disorder (seizures or psychosis)
M- Malar Rash
D- Discoid Rash
** DDx requires 4 out of 11 criteria!!!
+ ANA : TQ
think SLE - more common in young women and nonwhites
in SLE see malar rash, anemia, leukopenia, thrombctyopenia, GN, CNS disease, splinter hemorrhages, synovitis, conjunctivitis, pleurisy, pleural effusion
systemic presentation of fever, anorexia, malaise, w/l
Anti-Jo- 1
polymositis/dermatomyositis
indicates worse chance of pulmonary fibrosis!
ANCA
Granulomatosis w/ polyangiitis (Wegeners granulomatosis)
anticentromere pattern
think CREST
peripheral rim anti-DNA
seen in SLE
where is alveolar hemorrhage seen?
think Lupus and Goodpasture’s syndromes
what are lung and heart involvements in SLE? TQ
Bronchopneumonia, pleural effusions, restrictive lung disease, alveolar hemorrhage*, coronary aneurysms, myocarditis, hypertension, CHF, and Libman-Sacks endocarditis**
an imp. drug to know in tx of SLE?
Hydroxychloroquine -
prevents flares, reduces congenital heart block, lowers diabetes risk, and reverses platelet activation by IgG antiphospholipid.
causes of splinter hemorrhages? TQ
- Nail trauma
- subacute bacterial endocarditis
- Antiphospholipid antibody syndrome – including lupus
- Hypereosinophilic syndrome
if found to have cardiac echo…. think Libman- Sacks vegetations
= (atypical verrucous vegetations) with
mitral regurgitation
= (Marantic or non-bacterial thrombotic
endocarditis)
Libman-Sacks endocarditis
a form of nonbacterial endocarditis that is seen in systemic lupus erythematosus. It is one of the most common cardiac manifestations of lupus
vegetations are small and formed from strands of fibrin, neutrophils, lymphocytes, and histiocytes. The mitral valve is typically affected, and the vegetations occur on the ventricular and atrial surface of the valve.
CAUSE MITRAL REGURG!
Which drugs can give drug induced lupus? TQ
Hydralazine Isoniazid Procainamide Methimazole Prophylthiouracil Etanercept
Will see no renal disease, no CNS disease, +anti-histone Abs
Slow acetylators: *if you inhibit DNA methylation in helper T cells, they lose requirement for antigen =auto-reactive.
(i.e. isoniazid)
which staining is seen in drug induced SLE? TQ
anti-histone “diffuse/homogenous” antibodies
Lupus and pregnancy: how do you tell she has a lupus flareup rather than preeclampsia? TQ
- should be screened for LA, antiphospholipid Ab, apolipoprotein H
- use aspirin to decrease risk of antiphospholipid Ab syndrome
SLE vs. preeclampsia:
- both have HTN, proteinuria, low platelets
- SLE has dsDNA, **low C3/C4
- preeclampsia: has abn LFT’s and high uric acid
most common Ab seen w/ annular lesion of SCSLE? what to worry about? TQ
AntiRO/SSA and AntiLa/SSB
- worry about heart block!!!!
Subcutaneous SLE:
- Spares knuckles and face
- scaling annular lesions
- Sun exposed areas
- Also seen in RA and SS
- Can be caused by HCTZ
** don’t want baby to get heart block **
neonatal lupus: TQ
** Affects children born of mothers with Anti – Ro (SSA) or La (SSB). **
Transient:
- rashes of SCSLE
- thrombocytopenia
- HA
- arthritis
- complete heart block **
hx: spontaneous abortions, TIA, DVT, see reticulated rash. LAbs show elevated INR and PTT, along with low platelet count? TQ
patient has lupus anticoagulant -> APS
causes APS (Anitphospholipid Syndrome)
APS = hypercoagulatbility w/ thomboses
- presents with thombocytopenia, but no other features of SLE
- may see DVT, PE, change in mental status, microangiopathic nephropathy
how to DDX APS? TQ
Antiphospholipid Antibody syndrome:
To diagnose need 1 clinical criteria plus 1 lab criteria 3 months apart.
Lab criteria:
1. Anticardiolipin antibody (AcL)
- Lupus anti-coagulant (LA)
- Anti-β2 glycoprotein 1* (ApoH**) (anti-B1GP)
Clinical Criteria: CLOTS!!!!
- Spontaneous abortion
- Vascular thrombosis: TIA, CVA, thrombosis, DVT, PE, Budd-Chiari syndrome, infarction, MI, heart valve disease, adrenal infarction… etc.
needs to be 3 mos apart!!!
confirm w/ russel viper venom test
Abs in APS?
Lupus anticoagulant is the worst one and assoc. w/ thrombotic events in APS
if have all three Abs = worst disease!
tx for APS? TQ
***Hydroxychloroquine (retinal screening) = reduces thrombosis.
hx: pt. w/ hep C presents with rash (livedo reticularis = giraffe rash)
giraffe rash = Spasm of the deeper arterioles
due to: cryoglobulinemia
Progressive Systemic Sclerosis Syndrome??
A syndrome characterized by densely packed collagen and fibrosis :(could be d/t metabolic defect with increased collagen in EC matrix…)
- Raynaud’s syndrome.
- Cutaneous, subcutaneous calcification, pigmentation
and depigmentation, telangiectasias. - Cardiac: pericarditis, fibrosis, MI*, heart block, R-CHF.
- Pulmonary: restrictive lung disease with decreased CO diffusion, pulmonary hypertension.
- GI: GERD, sticking below the sternum, hypomotility with bacterial overgrowth, large mouth diverticulum.
- sticking below sternum, d/t
- Renal: “onion skinning” and hypertension.
NOTE CREST, is part of scleroderma (“limited scleroderma” )
how to ddx scleroderma? TQ
= Fibrosis and vasculopathy of the skin and visceral organs.
1 major or 3 minor criteria necessary:
Major:
1. symmetric thickening, tightening, and induration of the skin proximal to the metacarpophalangeal (or MTP) joints , ie. arms, trunk, etc.
Minor:
- sclerodactyly (swelling and flexion of fingers)
- digital pitting
- bibasilar pulmonary fibrosis
in full blown scleroderma will hear “tendon friction rubs” over the forearms and shins!
CREST
can ddx high probability of scleroderma w/ 3/5 CREST sx
= “limited scleroderma”
Calcinosis, Raynaud’s, Esophageal dysmotility, Scleroderma, telangiectasias
DDx of Raynauds? TQ
Raynauds = episodic ischemia of digits, ears or nose w/ triphasic color changes: pallor, cyanosis, rubor
** Primary RP: age < 30, symmetrical, normal SR, no nail bed capillary changes.
Secondary RP:
- CT disease, especially scleroderma with nail capillary changes and serological changes.
- Drugs & Toxins – bleomycin
- Structural arterial disease – atherosclerosis, emboli, migraine.
- Occupational –vibration/jackhammers.
- Hemorrheologic - PV, cryoglobulins, cold agglutinins.
- Other - thoraic outlet and carpal tunnel syndrome,
- Pheochromocytoma, hypothyroidism.
NOTE: need to know how to seperate primary from secondary b/c secondary raynauds is often the first sign of scleroderma!
what are visceral changes in scleroderma?
Risk of internal organ involvement strongly linked to extent & progression of skin thickening.
Involvement of internal organs, including heart, lungs, GI tract, and kidneys, accounts for increased morbidity and mortality.
- hypomotile bowels: bacterial overgrowth and atrophic tubules
- pulmonary fibrosis
- heart block
- diastolic dysfunction
- kidney involvement: HTN, this is what eventually kills a person
miracle drug that helps tx scleroderma?
ACEI’s!!!
before this drug, renal disease used to be #1 killer in scleroderma
what Ab is associated with sclerodermal renal disease? TQ
Risk factors pointing to renal disease:
** anti-RNA Polymerase III** (“speckled ANA”) - goes along with onion skinning and renal failure
rapid skin progression
anemia
( May present as hypertension, encephalopathy, sudden decrease in GFR, RBC or protein in urine, grade 3 to 4 retinopathy, or even micoangiopathic hemolytic anemia. )
sclerodermal cardiac disease?
Often silent pericardial effusions, LV diastolic dysfunction, conduction abnormalities, arrhythmias, myocarditis, microvascular disease (with possible MI) and myocardial fibrosis.
What Ab is associated with scleroderma lung disease? TQ
Interstitial Lung Disease
**Anti-topoisomerase (Anti-SCL-70) = worsening lung disease
= leading cause of scleroderma death!
what lung disease and Ab seen more w/ CREST? TQ
higher incidence of pulmonary HTN
risk factors:
- telangiectasia
- *** + anticentromere ab
causes of dysphagia?
SAD CREaP
SAD = solid and liquids
- scleroderma, achalasia, diffuse esophageal spasm
CREaP =
Carcinoma
Ring(Schatski’s)*/webs**
Eosinophilic esophagitis
and
Peptic stricture
nephrogenic fibrosing dermopathy? TQ
looks like scleroderma
- caused by CT use of gadolinum
- no autoabs
- affects skin, mm, heart, kidneys
(lacks raynauds, sclerodactyly, nail capillary changes)
eosinophilic fascitis? TQ
scleroderma like syndrome
follows vigorous exercise - just see one area of fibrotic thickening of fascia d/t eosinophilia
assoc. w/ M protein
see “groove sign” - inflamm. and fibrosis in subcu tissues yield a “woody texture” of distal limbs
Plantar/Palmar Fascitis and Polyarthritis Syndrome
Paraneoplastic syndrome: adenocarcinomas and hematologic malignancies (MM, CML, Hodgkins)
- fibroblast proliferation d/t TGF-beta
- thickening of palmar fascia
- finger flexion contraction
three types of inflammatory mm. diseases? TQ
- Polymyositis – painless proximal muscle weakness (difficulty in arising from a chair, climbing stairs, or lifting arms above the head).
- Dermatomyositis – painless proximal muscle weakness with preceeding rash.
- Inclusion Body Myositis - asymmetric distal weakness, thigh atrophy, finger flexor weakness, dysphagia, and older men.
what do you need to ddx Dermato/Polymyositis: TQ
- Shoulder and hip weakness
- Elevated enzymes
- Positive EMG
- Myofiber degeneration with mononuclear infiltration
- Skin changes in DM (shawl sign, Gottron’s papules, heliotrope eyes, and nailfold capillary loops) - seen only in dermatomyositis
3/5 suggest DM/PM
4/5 define the diagnosis
pathogenesis of DM/PM
DM = B cells PM = CD8 T cells
DM: Autoimmune microangiopathy. Complement activation with MAC and lysis of endomysial capillaries, capillary necrosis, perivascular inflammation, muscle ischemia, and muscle fiber destruction. Infiltration of B cells, CD4 cells and upregulation of MHC 1 and adhesion molecules. = B cell disease
PM and IBM: Cytoxic CD8 T cells with perforin and macrophages invading myosites in endomysial areas. = T cell disease
characteristics of dermatomyositis?
Women:Men / 2:1. 40s and 50s.
Autoimmune HLA DR 3,5,7, DRW52.
Symmetrical proximal muscle weakness.
Difficulty climbing stairs or arising from chair.
Oropharyngeal (proximal) dysphagia. (b/c skeletal mm. is problem)
Facial erythema.
Shawl sign.
Heliotrope (purple rash around eyes)
Gottron’s patches (over nuckles)
Mechanic’s hands.
** may get puomonary/cardio involvement: ILD, CHF
