What does sensitive mean?
true +; few false -; indiv has disorder
What does specific mean?
true - ; few false +; excludes indiv with disorder
What is the 1st titer test given for autoimmune disorders?
ANA (antinuclear antibody)
What rules out a diagnosis of SLE in regards to the ANA test?
a negative titer
Is the ANA test sensitive or specific for detecting lupus?
sensitive (higher the titer = greater likelihood of disease/ if positive need to do other antibody testing)
What happens if the ANA test is positive but the titer is at a lower level?
think leukemia, mono, myasthenia gravis, cirrhosis, chronic hepatitis
What is the most specific test for diagnosing SLE?
Anti-native DNA (especially with high titers)
What would your anti-native DNA level be if you have lupus?
positive > 200 U (negative < 70 U; borderline 70-200 U)
What if you have a low to intermediate anti-native DNA titer?
other rheumatic disease, chronic hepatitis, mono, biliary cirrhosis
What is a positive rheumatoid factor test found in a dilution of greater than 1:80 seen in?
SLE, scleroderma or sjogren's (but - does not exclude the dx of RF/ elderly also have false positives)
What immunoglobulins make up RF?
IgM, IgG and IgA which cause immune complex activation leading to joint pain
What is the anti-SM test used to diagnose?
SLE or r/o other mixed connective tissue damage/ immunoglobulin specific against Sm
What happens if anti-SM is positive? negative?
positive = lupus present and CNS involvement
negative = lupus is not ruled out
What do the Anti-SS-A and Anti-SS-B tests diagnose?
Is the Anti-SS-B test or Anti-SS-A test more specific for Sjogren's?
Anti-SS-B (only in Sjogren's/ Anti-SS-A also in some people with SLE)
What is the Anti-SCL-70 test used to diagnose? What pattern does it have?
scleroderma with a speckled pattern
What does the ANCA test help you diagnose?
Wegener's granulomatosis; follow the course and monitor the response to therapy
What does the C-ANCA test show in regards to WG?
highly specific for WG if limited to the kidney; may not be positive if WG is inactive
What does the P-ANCA test show in regards to WG?
found in 50% of pts with dz localized to kidneys, IBD and autoimmune hepatitis
What is a chronic inflammatory disease that can affect any organ system?
SLE aka systemic lupus erythematosus
When and in who is SLE in?
AA, Hispanics and Asians; dx in early 20-30's; female: male = 10:1
What are the highest causes of morbidity and mortality in SLE from?
What is the pathogenesis of SLE?
body sees something as foreign --> abnormal immune response --> autoantibodies and immune complexes activate complements --> inflammation (symptoms) --> irreversible organ damage over time
What are the criteria for lupus?
malar rash, discoid rash, photosensitivity, oral ulcers, arthritis, serositis, renal disorder, neurologic disorder, hematologic disorder, immunologic disorder, antinuclear antibody
What are the 4 features of drug-induced lupus-like syndrome?
HENN: (1) Hypocomplementemia & antibodies to native DNA = absent (2) Equal Sex Ratio (3) Nephritis & CNS features = absent (4) Normal Labs & Clinical Features once drug removed
What drugs is drug-induced lupus-like syndrome usually from?
hydralazine, quinidine and procainamide, phenytoin, isoniazide, d-penicillamine
What is the earliest symptom of lupus that 90% of pts have?
What are skin manifestations of lupus?
butterfly or malar rash, discoid rash, splinter hemorrhages, naif fold infarcts, alopecia
What are S/S of lupus?
fatigue (90%), fever, anorexia, malaise, wt loss
What are other manifestations of lupus?
ocular, hematologic, renal, CNS, pulmonary, cardiac, GI
What test is sensitive but not specific for SLE?
What level does the anti-native DNA test need to be to diagnose lupus?
What tests are specific but not sensitive for lupus?
anti-double stranded-DNA and anti-Sm
What does the anti-double stranded DNA test mean in regards to lupus?
What does the anti-Sm test mean in regards to lupus?
What are the ANA patterns associated with lupus?
outline pattern (only with SLE), homogeneous or speckled
What should you do if you have a positive ANA with a homogeneous or speckled pattern? Or if you have a negative ANA with signs and symptoms of SLE?
do an anti-ds DNA and an anti-Sm
What are the drugs used for treatment of lupus?
DaMN PaW: Danazol (refractory thrombocytopenia), Mofetil (immunosuppressants), NSAIDs (antiinflammatory), Plaquenil (hydroxychloroquine, anti-malarial & skin problems), Warfarin (clotting & antiphospholipid syndrome)
Does lupus have a favorable or unfavorable prognosis?
A pt comes in with mental status changes, recent thrombosis, Livedo reticularis, skin ulcers and cardiac valvular dysfunction. What do they have?
What is the essential of dx for antiphospholipid syndrome?
hypercoagulability with recurrent thromboses in either venous or arterial circulation/ thrombocytopenia = common
In which patients is Antiphospholipid syndrome primarily diagnosed?
pts with recurrent venous, arterial occlusions or thrombocytopenia without other SLE features/ present in 20-30's in people with acute MI or stroke/ mostly with other autoimmune disorders
When is antiphospholipid syndrome asymptomatic until?
1st thrombolytic event (i.e. DVT, PE, CVAs)
What are the 3 types of antiphospholipid antibodies associated with antiphospholipid syndrome?
LAA: Lupus anticoagulant; Anti-cardiolipin antibody; Antibody associated with biologic false-positive test for syphilis
What must you rule out if you are unsure about antiphospholipid syndrome in a young pt with stroke and no other risk factors?
protein C, protein S and antithrombin III deficiency
What is treatment of antiphospholipid syndrome?
anticoagulant with warfarin with INR of 2.5-3.5
What test is positive in regards to antiphospholipid syndrome?
Anti-B2 glycoprotein I
What disease is common in pts with autoimmune disorders or liver dysfunction? It is also a chronic disorder characterized by diffuse fibrosis of the skin and internal organs.
What may affect large areas of skin and organs such as the heart, lungs, or kidneys; fibrotic changes of the lung?
What 2 aspects of scleroderma are in almost all pts?
Raynaud's phenomenon and antinuclear antibodies
What is an essential of dx for scleroderma?
diffuse thickening of skin with telangiectasia
What are systemic features of scleroderma?
dysphagia, hypomotility of the GI tract, pulmonary fibrosis, cardiac & renal involvement
What pattern is the ANA in association with scleroderma?
nuclear or speckled
Which form of scleroderma is in more people and has better outcomes?
limited (in 80%)
What are the symptoms of limited scleroderma?
CREST:calcinosis cutis (ca deposits affecting mov'ts), Raynaud's phenomenon, esophageal motility d/o, sclerodactyly & telangietasia; limited to face and hands; rarely develop renal failure or lung dz
What is the other form of scleroderma in fewer people? What are its symptoms?
diffuse (20%); skin changes including the trunk & proximal extremities, tendon friction rubs over forearms & shins, cardiac dz
What scleroderma symptoms are in 90% of pts?
polyarthralgia and Raynaud's
What are lab findings of scleroderma?
mild anemia, elevated ESR, proteinuria, Anti-SCL-70 (specific), ANA +
What are the treatments for Scleroderma?
CHAANCe: CCB's for Raynauds ( nifedipine 30-120mg/d)
GERD: avoid late night meals, elevation of bed, H2 Blockers & antacids
-HTN caused by renal crisis= ACE I( captopril 37.5 mg/d in 3 doses)
A pt comes in with burning, itching and sensation of a foreign body in their eye. They have a large tongue and a dry mouth, photophobia, denatal carries, nose dessication. What do they have?
What are systemic symptoms of Sjogren's syndrome?
Dysphagia, vasculitis=m/c assoc. w/ antiphospholipid syndrome, pancreatitis= common, obstructive lung dz w/ fibrotic changes
What are lab findings of Sjogren's syndrome?
Anti-SS-A(in secondary) and Anti-SS-B (more specific, in primary), mild anemia, leukopenia, polyclonal hypergammagobulin (all elevated), RF 70%, ANA 95%, enlarged lymph nodes & higher risk of lymphoma
What are the treatments for Sjogren's syndrome?
SAP: sugar free-gum(dry mouth), artificial tears (dry eyes) pilocarpine 5mg qid (pill form of eye drops)
*avoid decongestants due to anti-cholinergic properties