Approach To Malaise And Generalized Pain Flashcards
What clinical markers are present in Lupus like syndrome/drug-induced
+ANA
+Anti histone antibody
What causes a drug induced SLE?
Sulfa drugs Hydralazine Isoniazid Minocycline TNF Inhibitors Quinidine Chlorpromazine Methyldopa Procainamide
What antibodies is neonatal lupus associated with?
Anti Ro or La Abs
What is the treatment for SLE?
Avoid sun
NSAIDS
Corticosteroids
Hydroxychloroquine
What causes mortality in the early years after diagnosis of SLE?
Later years?
Infections - opportunistic
Kidney or CNS
Accelerated atherosclerosis - MI
Thromboembolic events
How do you manage SLE patients?
Minimize other conventional risk factors for atherosclerosis
Avoid smoking
Flu vaccines
Pneumococcal vaccines
Cancer screenings (increased risk of malignancy)
Consider avascular necrosis of the bone due to corticosteroid use
What type of hypersensitivity is SLE?
TYPE III
What is the treatment of discoid lupus?
Photo protection + topical anti-inflammatory agents of systemic antimalarial drugs
What is the hallmark of scleroderma?
Thickening and hardening of the skin (fibrosis of the skin and visceral organs)
What causes the dry itchy skin in scleroderma?
Obliteration of eccrine sweat and sebaceous glands
What are the three forms of scleroderma?
Diffuse
Limited (Crest syndrome)
Localized
What is a manifestation of limited scleroderma?
Pulmonary HTN
What is a manifestation of diffuse scleroderma?
Interstitial lung disease
What does CREST stand for?
Calcinosis cutis Raynauds Esophageal dysmotility Sclerodactylyl Telangectasia
Is renal crisis common in limited scleroderma?
No
What is the primary cause of morbidity and mjortality in scleroderma?
Pulmonary disease
Interstitial lung disease seen in diffuse
Pulmonary artery hypertension seen in limited
How do you diagnose PAH?
A mean pulmonary arterial pressure of 25 mmHG OR GREATER
How do you confirm diagnosis of PAH?
Right heart catheter
Which cancer shows an increased incidence in scleroderma?
Bronchoalveolar carcinoma
Esophageal adenocarcinoma
What is the diagnostic work up for scleroderma?
Blood pressure ESR ANA UA CXR Barium swallow Echo PFT Skin biopsy Serology
What is the serology for Scc (diffuse and limited)?
Diffuse - anti Scl 70, anti RNA polymerase III
Limited - +anti centromere
How do we treat Scc?
Treat the symptoms
CCB ACE inh Anti reflux Glucocorticoids Cyclophosphamide PDE inh
What is an adverse affect of high dose corticosteroids?
Renal crisis
What is the limited Scc characterized by?
Benign skin condition of discreet areas of discolored skin induration
Morphea (patches)
What are the manifestations of sjogren?
Dry eyes Dry mouth Vaginal dryness Tracheobronchial dryness Parotid enlargement
What is keratoconjunctivitis sicca?
Inadequate tear production causing a foreign body sensation
What cancer is sjogrens associated with?
B Cell NHL (Maltoma)
What is the serology work up for sjogrens?
\+anti SSA/Ro \+anti SSB/La Low C4 Hypergammaglobulinemia High ESR \+RF \+ANA
Treatment of sjogrens?
Treat symptoms
Eye lubricants
Frequent water
Hydrdoxychloroquine
Glucocorticoids
Avoid atropine drugs and decongestants
how do you characterize the muscle involvement in inflammatory myopathies?
Symmetrical proximal muscle weakness
What are the four key skin features of dermatomyositis?
Gottron’s patches
Heliotrope rash
Periungual erythema
V neck Erythema
What is the muscle biopsy finding in dermatomyositis?
Perimysial and perivascular inflammation, perifascicular atrophy
What is seen in labs for Dermatomyositis?
Elevated CK and aldolase
What is seen on serology of dermatomyositis?
Anti Jo 1
Anti Mi2
Anti MDA5
Anti P155/P140
What malignancies are associated with dermatomyositis?
Ovarian (check CA 125) Lung Pancreatic Stomach Colorectal NHL
What is seen on the biopsy for polymyositis?
Endomysial inflammation with invasion of non necrotic muscle fibers
What is seen on labs for polymyositis?
Elevated CK
What is seen on serology for polymyositis?
Anti Jo1
What is the treatment for dermatomyositis and polymyositis? **
Corticosteroids
What is the characteristic muscle weakness in inclusion body myositis?
Finger flexion or quadriceps weakness
What is seen on biopsy of inclusion body myositis?
Endomysial inflammation, rimmed vacuoles, invasion of non necrotic muscle fibers
What is seen on labs of IBM?
Elevated CK
What is seen on serology of IBM?
Anti cN1A autoantibodies
What is the treatment of IBM?
Supportive
How do you diagnose Henoch schonlein purpura?
IgA deposits on biopsy
How do you treat HSP?
Glucocorticoids
How do you diagnose Goodpastures?
Biopsy shows anti basement membrane autoantibodies in basement membrane
What causes the hypertension in Takayasu arteritis?
Renal artery stenosis
How do you diagnose takayasu arteritis?
MRI or CT angiography
Biopsy shows granulomas with giant cells
How do you treat takayasu arteritis?
Glucocorticoids
What is the triad in Behcet syndrome?
Recurrent mouth ulcers
Genital ulcers
Uveitis
What HLA for Behcet syndrome ?
HLA B51
What is the treatment of Behcet?
Low dose GC
What is polyarteritis nodosa characteristically assoc. with?
Hep B virus
What is seen on biopsy for PAN?
FIBRINOID NECROSIS
What is seen on serology for PAN?
P ANCA
Tx of PAN ?
Corticosteroids
What do patients die from in Kawasaki disease?
Coronary involvement
Aneurysm or MI
What is the tx of Kawasaki disease?
IVIG and high dose ASA
What is seen on serology for Wegeners granulomatosis?
C ANCA
What is the hallmark of Wegeners?
Granulomatous inflammation
What is the tx of Wegeners?
Cyclophosphamide and high dose GC or rituximab
What are the hallmarks of Churg Strauss syndrome?
Asthma + eosinophilia -> vasculitis with granulomas
How do you make the diagnosis of Churg Strauss?
MPO ANCA
How do you treat Churg Strauss?
GC
Who is affected by Buerger disease?
Smokers
How do you make the diagnosis of Buerger disease?
Angiography corkscrew appearance
Treatment of Buerger disease?
Smoking cessation
How does temporal(giant cell) arteritis present?
Headache, jaw claudication, visual abnormalities (amaurosis fugax or diplopia)
What HLA is associated with temporal arteritis?
HLA DR4
How do you diagnose temporal arteritis?
Temporal artery bx
1.0 cm segment biopsy will show segmental granulomatous vasculitis with multinucleated giant cells
How do you treat temporal arteritis?
Corticosteroids therapy before biopsy
What is polymyalgia rheumatica (PMR) associated with?
Temporal/giant cell arteritis
How do you diagnose PMR?
Elevated ESR and CRP
How do you treat PMR?
Corticosteroids
How do you diagnose secondary raynauds?
Nailfold capillaroscopy
What serology marker can we use to identify an autoimmune disease?
+ANA
What is the clinically significant ratio of ANA?
1:160
What are the four ANA patterns?
Homogenous
Speckled
Centromere
Nucleolar
What staining pattern does CREST show?
Centromere pattern
Which staining pattern does drug induced SLE show?
Homogenous
Which staining pattern does Sjögren’s syndrome show?
Speckled mostly but can also be homogenous
What staining pattern does diffuse systemic sclerosis show?
Nucleolar pattern (more than the others) can also be speckled
What do the sx of malaise, malar rash, photosensitivity, symmetric inflammatory arthritis, thrombosis, pancytopenia, pericarditis, seizures and psychosis indicate ?
SLE
Antibodies in SLE include what? (3)
ANA, anti-ds DNA, anti Sm (smith)
Which antibody present in SLE correlates with disease activity?
Anti-ds DNA
What happens to complement levels in SLE?
Decreased C3 and C4
Which type of APA syndrome can cause biological false positive tests for syphilis?
Type one
Which type of APA syndrome can cause lupus anticoagulant and is a risk factor for venous and arterial thrombosis and miscarriage ?
Type two
Which type of APA syndrome can cause anti cardiolipin antibodies?
Type three
What are anti cardiolipin antibodies directed at?
Beta 2 GPI
What is the treatment for APA syndrome?
Anticoagulation