Term
| What AB's are present in polymyositis? |
|
Definition
| Anti Mi 2 AB and Anti Jo 1 AB |
|
|
Term
| What AB's are present in Drug-Induced Lupus? |
|
Definition
| Anti-histone AB (Is present when a person has a rxn to meds--> specifically Procainimide, hydralizine, sulfas, quinidine) |
|
|
Term
| What AB's are present in Systemic Lupus Erythematosus (SLE)? |
|
Definition
Anti-SSA (Ro)/Anti-SSB (La) AB
Anti-ds DNA AB
Anti-smith AB (HIGHLY specific for systemic lupus) |
|
|
Term
| What AB's are present in Sarcoidosis? |
|
Definition
| Angiotensin Converting Enzyme |
|
|
Term
| What AB's are present in Subcutaneous Lupus Erythematosus/Discoid Lupus? |
|
Definition
| Anti-SSA (Ro)/Anti-SSB (La) AB |
|
|
Term
| What AB's are present in Polyarteritis Nodosa? |
|
Definition
| p-ANCA (perinuclear is non-specific) |
|
|
Term
| What AB's are present in Mixed Connective Tissue Dz? |
|
Definition
| Anti-RNP AB (HIGHLY specific for MCTD) |
|
|
Term
| What AB's are present in Progressive System Sclerosis? |
|
Definition
Anti-SCL 70 AB- PSS is diffuse scleroderma
*Presence of this AB leads to a BAD prognosis--> will lead to progressive dz, HIGHLY specific for PSS |
|
|
Term
| What AB's present in Neonatal Lupus? |
|
Definition
| Anti-SSA (Ro)/Anti-SSB (La) AB--> RO SPECIFIC, La may be present |
|
|
Term
| What AB's are present in RA?? |
|
Definition
| Anti-CCP AB (VERY specific for RA) |
|
|
Term
| What AB's are present in Sjogren's Syndrome? |
|
Definition
| Anti-SSA (Ro)/Anti-SSB (La) AB |
|
|
Term
| What AB's are present in Wegener's Granumlomatosis? |
|
Definition
| c-ANCA (cytoplasmic is highly specific for Wegener's) |
|
|
Term
| What AB's are present in CREST? |
|
Definition
| Anti-centromere- CREST is limited scleroderma (HIGHLY specific for CREST) |
|
|
Term
| What is discoid lupus? Will ANA be negative or positive? How is it dx? |
|
Definition
| Localized, SCARring dz seen usually on the face, cheeks, scalp, arms and upper chest. ANA may or may not be negative (NOT specific). Dx by lupus skin bx (lupus-band test). |
|
|
Term
| What is the lupus band test? |
|
Definition
| Immune complexes deposited at the dermo-epidermal junction are bx and stained with immunoflorescence for the presence of antibodies. |
|
|
Term
| What is the initial systemic therapy of choice when cutaneous lupus cannot be controlled (progresses to SLE)? |
|
Definition
Plaquenil (Hydroxychoroquine) (antimalarial)- Major side effect is irreversible retinopathy.
*If pts do not respond to this, chloroquine can be used** |
|
|
Term
| What is Subacute Cutaneous Lupus Erythematosus (SCLE)? |
|
Definition
| Annular, circular, or papulosquamous lesion that is NON-SCARRING. Pts are photosensitive (involves exposed areas) |
|
|
Term
| What is the treatment for SCLE/Discoid Lupus? |
|
Definition
1. Topical steriods*
2. Plaquienl (Hydroxychorquine)*
3. Intralesional steroid injection (Kenolog)
4. Thaliodmide (tetratrogenic effect)
5. Leflunomide
6. Sun Block |
|
|
Term
| How is neonatal lupus transmitted? |
|
Definition
| Vertical transmission- Mother has SSA (Ro+) antibodies that travel across the placenta (sometimes assoc with SSB (La+) IgG) |
|
|
Term
| How long does neonatal lupus last? What are the effects of neonatal lupus? |
|
Definition
It is transient (baby is born with rash and it resolves in 8 months once mom's AB disappear).
Effects: AB bind to fetal cardiac tissue and cause heart block with bradycardia and/or myocarditis; thrombocytopenia, leukopenia, rash are also common |
|
|
Term
| What are the definite drugs that trigger drug-induced lupus? What drugs may trigger it? What Anti-TNF inhibitors may trigger it? |
|
Definition
Definite: Hydralazine, Minocin and procainamide
Possible: Quinidine, phenytoin, sulfas, D-Penicillamine and Isonizaid
Anti-TNF (Biologics): Remicade, Enbrel, Humira |
|
|
Term
| Describe the course and side effects of drug-induced lupus. Tx? |
|
Definition
Self-limiting. Main SE: Serositis.
Tx: Removal of agent will cause sx to reside. |
|
|
Term
In Drug induced Lupus, describe the following:
1. ANA
2. Complements
3. Anti-DNA AB |
|
Definition
1. Slower to resolve
2. Normal
3. Negative |
|
|
Term
| Pts with SLE have which type of complement deficencies? |
|
Definition
| C4 allele def (does not do well against encapsulated organisms) |
|
|
Term
| What agents trigger lupus? |
|
Definition
| Sunlight, diet, infxns, stress, meds (Sulfa AB, hormones, OCs) |
|
|
Term
| What is the pathogenesis of SLE? |
|
Definition
| Excessive T Helper cells with low T suppressor cell activity which results in B cell proliferation and auto-AB production |
|
|
Term
| What are the clinical features/classification criteria for SLE? |
|
Definition
"DOPAMINE RASH"- 4/11 Needed
D- Discoid Lupus
O- Oral aphthous ulcers
P-Photosensitivity
A-Arthritis/Synosvitis (usually hands, wrists, knees)
M-Malar Rash (spares naso-labial, absence of discrete papules or pustules distiguishes it from acne rosacea)
I- Immunologic- Anti-ds-DNA, anti-smith, anti Ro/La
N- Neurologic: psychosis, personality change, seizures
E- ESR Increases (frequent lab finding)* not part of criteria
R-Renal dz: nephritic/nephrotic syndrome
A-ANA +
S- Serositis (pleursy, pericarditis)
H-Hematologic: hemolytic anemia, leukopenia, thrombocytopenia |
|
|
Term
| What AB correlates with lupus nephritis? |
|
Definition
|
|
Term
| What will labs show with SLE? |
|
Definition
1. Leukopenia, usually lymphoma
2. Anemia (Normochromic/Normocytic or hemolytic)
3. Thrombocytopenia
4. Hypergammaglominemia
5. ANA +
6. Complement- Low C2, C4, CH50 |
|
|
Term
| What drug is used to tx joint pain, skin rash and decrease flares and steriod use in SLE? |
|
Definition
| Plaquinel (Hydroxychoroquine) (DOC- not as toxic as methotrexate) |
|
|
Term
What sx are the following drugs used to tx in SLE?
1. Methotrxate
2. Leflunomide
3. Cyclophosphamide
4. Azathiprine
5. Steriods |
|
Definition
1. Arthritis
2. Arthritis, rash and major organ involvement
3. Serious renal dz or vasculitis
4. Serious major organ involvement
5. Arthritis or serositis or major organ involvement |
|
|
Term
| What are the major concerns in a person with Antiphospholipid Antibody Syndrome (APA or Anti-Cardiolipin ABS) ? |
|
Definition
1. *Thromboses- venous or arterial
2. Spontaneous Abortions
3. Thrombocytopenia
4. Livedo reticularis: fish-net, bluish discoloration of skin (not specific but assoc.) |
|
|
Term
How do you tx a pt with APA and NO thrombosis?
APA with thrombosis? |
|
Definition
Without: Baby ASA (81 mg)
With: Coumadin with HIGH INR btwn 3-4
*Exceptions: No anticoag if platelets <50,000 |
|
|
Term
| What is Scleroderma? Onset? |
|
Definition
Chronic multi-system dz characterized by fibrotic changes in the skin, lungs, GI and renal systems with widespread small vessel vasculopathy.
Onset: 30-50 Women (MC) |
|
|
Term
| What is the correlation btwn anti-smith AB and prognosis in pts with SLE? |
|
Definition
| The more anti-smith AB, the WORSE the prognosis. |
|
|
Term
|
Definition
Limited cutaneous scleroderma characterized by:
C- Calcinosis: rock hard subQ deposits
R- Raynaud's Phenomena (5-10 yrs b/f sx):
E- Esophageal Dysmobility
S-Sclerodactyly- claw fingers, scar hands
T- Telangiectasias (BLANCH) |
|
|
Term
|
Definition
1. Anti Centromere AB
2. Raynaud's ALWAYS present
3. Sclerodactyly to wrists (BUT NOT beyond wrists) |
|
|
Term
| Describe the cutaneous changes of CREST. |
|
Definition
1. Early diffuse swelling of fingers (boxer glove appearance)
2. SLOW progression (prognosis better than PSS)
3. Involves hands below wrist and mouth
4. Telengectasias
5. SubQ Ca- seen at sights of trauma. |
|
|
Term
| How is PSS different from CREST? |
|
Definition
| PSS has diffuse skin and major organ involvement. Raynaund's is NOT always present at first, but quickly follows. |
|
|
Term
| What are the cutaneous changes of PSS? |
|
Definition
*Early diffuse swelling/stiffness of fingers and rapid progression are the most notable.
Also- skin is involved beyond the wrists and includes trunk.
It's RARE to see telengectasias or subQ Ca |
|
|
Term
|
Definition
| A vascular disorder that affects blood flow to the extremities which include the fingers, toes, nose and ears when exposed to temperature changes or stress. |
|
|
Term
| What are the cyclic color changes of Raynaud's Dz? |
|
Definition
White (pallor)- becomes cold and numb
Blue (cyanosis)- episodic and when it subsides, or the area is warmed, blood returns to the area and the skin color turns.
Red (Rubor)
Normal- often accompanied by swelling and tingling and usually due to reactive hyperemias in areas of deprived blood flow. |
|
|
Term
| What is the difference btwn primary and secondary Raynaud's? |
|
Definition
Primary ("Allergic to Cold")- Sx occur by themselves in young women and are NOT accompanied by other dz for more than 4 yrs.
Secondary- Due to an underlying dz and is the initial sx in 90% of scleroderma pts. Usually progresses to necrosis and gangrene of the fingertips |
|
|
Term
| What factors can contribute to Raynaud's phenomenon? |
|
Definition
"BADCT"
B- Blood disorder (polycythemia)
A- Arterial (blockage)
D- Drugs (BB)
C- Connective Tissue Disorders (SLE, SCL)
T- Traumatic (Vibration Injury- jack hammer, construction workers, etc) |
|
|
Term
| If a person has PSS and Raynaud's is not initially present at onset, what does this mean? |
|
Definition
| There is an assoc with poor survival and higher incidence of renal and heart involvement. |
|
|
Term
| What is the tx of choice for an individual with Raynaud's assoc with CREST? |
|
Definition
| Ca Channel Blockers- they are most effective in controlling vasospasm via vasodilation. |
|
|
Term
| What are the musculoskeletal changes assoc with PSS? How are these tx? |
|
Definition
Polyarthralgias, friction rubs (VERY POOR prognosis), flexion contractures and myopathy
tx: NSAIDs or Plaquenil for synovitis |
|
|
Term
| What drug should never be given to scleroderma pts? |
|
Definition
| Steriods! These will exacerbate sx!! |
|
|
Term
| What renal complication is of greatest concern in PSS pts? How would you tx? |
|
Definition
Malignant arterial hypertension- need to watch for changes in BP!
tx- ACEi are very good in preventing renal crisis. |
|
|
Term
| What are the pulmonary conditions assoc with PSS? |
|
Definition
| Pulmonary fibrosis, restrictive dz (lungs scar, therefore do not expand normally), usually bibasilar |
|
|
Term
| When does pulmonary hypertension occur in CREST? What drugs does it respond to? |
|
Definition
Pulm HTN occurs late in the dz. Prognosis is poor bc pt will ultimately develop cor pulmonale.
Tx- CCB and bosarten |
|
|
Term
| How does PSS affect the GI system? |
|
Definition
| Pts will get esophagitis/reflux; distal motor dysfuction; small bowel and colon hypomobility with malabsorption and pseudo-obstruction; gasteroparsis bc parastalsis has ceased due to scarring. |
|
|
Term
| What are the most common GI conditions assoc with CREST? |
|
Definition
Primary biliary cirrhosis, esophagitis/reflux and distal motor dysfunction.
*It is uncommon for CREST to involve the lower GI. |
|
|
Term
| PSS usually involves the increase of which immunoglobulin? |
|
Definition
|
|
Term
| What is the etiology of Mixed CT dz? |
|
Definition
| Mixed CT Dz's are assoc with other disorders. When followed over time they evolve or branch out into a specific disorder such as SLE, PSS, RA etc. |
|
|
Term
| What are the 2 major characteristics of Mixed CT Dz? |
|
Definition
1. Raynaud's phenomenon
2 + anti- RNP AB
* When pts are + for the above 2 criteria: it is not definite at this point which path they will follow. You need to do supportive care until the actual condition develops |
|
|
Term
| What is relapsing polychondritis? |
|
Definition
| A severe, episodic and progressive inflammatory condition involving cartilage of the ears, nose and laryngotracheaobronchial tree. Can also include the eyes, CV system, peripheral joints, skin, middle/inner ear and CNS |
|
|
Term
| What are the cardinal signs of relapsing polychondritis? |
|
Definition
Localized auricular pain, erythema and swelling that spares the lobule bc there is no cartilage there.
*Also- episcleritis and saddle nose deformity |
|
|
Term
| What is the pathogenesis of relapsing polychonritis? |
|
Definition
1. Humoral- circulating AB to cartilage-specific collagen
2. Cellular- CD4 and natural killer T cells, IL-8 and various proinflammatory cytokines elevated |
|
|
Term
| What AB is assoc with relapsing polychonditis? |
|
Definition
|
|
Term
| What is the tx for relapsing polychronditis? |
|
Definition
Systemic corticosteriods- Prednisone is administer in the acute phase and is tapered for maintenance
Also- MTX, dapsone, Imuran and Alpha TNF inhibitors (infliximab) |
|
|
Term
| What are the most frequent causes of death assoc with relapsing polychondritis? |
|
Definition
1. Infection secondary to corticosteriod tx
2. Respiratory compromise
3. Systemic vasculitis
4. Malignancy unrelated to relapsing polychonditis |
|
|
Term
|
Definition
| Neutrophilic infiltration of the subQ fat beneath the dermis and epidermis. |
|
|
Term
| Which disorder is associated with dry eyes, dry mouth (sicca), is more common in older women, and is not assoc. with any other dz? |
|
Definition
| Primary Sjogren's Syndrome |
|
|
Term
|
Definition
| In panniculitis, lipocytes are organized into lobules by fibrous septa which contain blood and reticuloendothelial cells. |
|
|
Term
| Secondary Sjogren's Syndrome is similar to primary in regards to sx of dry eyes and dry mouth. BUT It is also assoc with? |
|
Definition
| Secondary Sjogren's Syndrome is assoc. with other Connective Tissue Diseases such as RA and SLE. |
|
|
Term
| The pathologic process of this disease is characterized by infiltration of lymphoid cells in the lacrimal and salivary glands? |
|
Definition
|
|
Term
| What is Weber-Christian Dz (Idiopathic lobular panniculitis)? |
|
Definition
| An inflammation (Predominantly PMNs) of subQ fat lobules that will always have skin lesion/rash (e. nodosum) involvement. |
|
|
Term
| Sjogren's Syndrome is diagnosed by what test? |
|
Definition
-Postive Schirmers Test (Paper in eyes and access the tear production)
-Positive Rose Bengal Staining (Conjunctival and corneal defects on slit lamp exam)
-Abnormal salivary gland or lip bx
-Postive RF, ANA, or SSA/SSB |
|
|
Term
| What sx are common in Weber-Christian Dz? Is there systemic involvement? |
|
Definition
Malaise, fever and arthralgias, N, V, abd pain, wt. loss, hepatomegaly.
Yes- internal organs can be involved- heart, lungs, intestines, spleen, kidney and adrenal glands. |
|
|
Term
| If an individual has Weber-Christian Dz with inflammation involving visceral organs, what is their prognosis? |
|
Definition
| These pts have significant morbidity and mortality |
|
|
Term
| What conditions can e. nodosum be seen in? |
|
Definition
1. Weber Christan
2. Sarcodiosis
3. Churig Strauss |
|
|
Term
| This condition can coalesce and is characterized by crops of raised tender nodules over pertibial surfaces (shins and ankles)? |
|
Definition
|
|
Term
| What is the tx for Weber-Christian Dz? |
|
Definition
1. Fibrinolytic agents
2. DMARDs- hydroxychoroquine, azathiprine, thalidomide, cyclophosphamide, tetracycline, cyclosporine, mycophenolate and clofazimine
3. Systemic steriods (Prednisone) may be effective in suppressing acute exacerbations
4. Nonsteriodal anti-inflammatory agents may reduce fever, arthralgias and other signs of malaise. |
|
|
Term
| If a pt has a red rash that DOES NOT BLANCH, what does this indicate? |
|
Definition
|
|
Term
| What is Henoch-Schonlein Purpura (HSP)? |
|
Definition
| Immunoglobulin IgA mediated small-vessel vasculitis predominantly affecting children (4-5) and young adults (<20). It often follows a URI, strep or use of meds (AB) |
|
|
Term
This ACR Criteria is specific for what disease?
1. Ocular symptoms = dry eyes
2. Oral= dry mouth
3. Ocular signs +Schirmer, Rose Bengal
4. +Salivary gland biopsy (lymphocytic infiltration)
5. Parotitis (Bilateral swelling)
6. Auto-antibodies (+anti-SSA, SSB abs) |
|
Definition
|
|
Term
| Classic triad of this disease includes: Xerostomia (Dry Mouth), Keratoconjunctivits sicca (Dry Eyes), Arthritis (Hot swollen, tender joints)? |
|
Definition
|
|
Term
| Necrotizing vasculitis characterized by fibrinoid destruction of blood vessels and leukocytoclasis (PMNs)? |
|
Definition
|
|
Term
| What are the clinical manifestations of Henoch-Schonein Purpura? |
|
Definition
| Palpable purpura, arthralgias/arthritis, abd pain, GI bleeding and nephritis |
|
|
Term
| What is the most serious complication of Henoch-Schonlein Purpura? |
|
Definition
| Progressive renal failure. |
|
|
Term
| What is the diagnostic criteria for Henoch-Schonlein Purpura? |
|
Definition
2 or more of the followng:
1. Pt <20 yo
2. Palpable purpura
3. Abd pain or GI bleeding
4. Extravascular or perivascular granulocytes on bx**
5. IgA deposition on tissue immunoflorescence** |
|
|
Term
| What is the Definitive Dx of Henoch-Schonlein Purpura? |
|
Definition
Skin bx, gut or kidney showing small vessel leukocytoclastic vaculitis and IgA deposition
(Not always ANCA +) |
|
|
Term
| What is the tx of Henoch-Schonlein Purpura? |
|
Definition
| Self limited to 4-16 wks therefore supportive therapy is necessary. Make sure pts get adequate hydration and replace any signs of excessive blood loss. |
|
|
Term
| Should steriods be used in Henoch-Schonlein Purpura ? With what sx would they be used in? With what sx would they not be used in? |
|
Definition
Should only be used if the dz looks as if it is not resolving. If pt has:
Glomerulonephritis: Systemic steriods would be used in order to protect the kidneys even though the occurrence of relapses may be more frequent.
GI pathology: Systemic steriods are NOT helpful and should be avoided |
|
|
Term
| What is Leukocytoclastic Vasculitis? |
|
Definition
| A very common hypersensitive, cutaneous vasculitis characterized by PALPABLE purpura mostly on the legs. It occurs in all ages and is brought on by infxns and meds. |
|
|
Term
| What are the treatment indications for Sjogrens? |
|
Definition
Salagen/Evoxac - salivary stimulant
Tear duct ablation/plugs
Plaquenil - helps to shrink lymphocyte invasion of glands
Steriods
NSAIDS |
|
|
Term
| Patients are at higher risk for what with Sjogrens? |
|
Definition
| Pts are at higher risk for lymphoma-check for LAD |
|
|
Term
| What is the pathogenesis of Leukocylastic Vasculitis? |
|
Definition
| Circulating immune complexes within small (sometimes medium) sized vessels with exudation of neurophils and sometimes fibrin around dermal venules, with extravasion of RBCs |
|
|
Term
| What is Sarcoidosis characterized by? |
|
Definition
| noncaseating granulomas in 2 or more organs |
|
|
Term
|
Definition
confirmed by finding a granuloma on x-ray and tissue bx.
Also, the ACE levels are elevated from the macrophages in the lungs |
|
|
Term
| What are the clinical features of Leukocytoclastic Vasculitis? |
|
Definition
| Palpable purpura, urticaria, bullae, ulcerations, neuromuscular, renal, GI, lung and any other organs with a rich vascular supply. |
|
|
Term
| what are the organ systems affected by sarcoidosis? |
|
Definition
| Pulmonary, dermatologic, ocular, musculoskeletal. |
|
|
Term
| What is the definitive dx for Leukocytoclastic Vasculitis? How do you tx? |
|
Definition
Skin bx!
Tx the underlying cause! |
|
|
Term
| What are the 3 stages of pulmonary sarcoidosis |
|
Definition
1. Bilateral hilar adenopathy
2. BL hilar adenopathy + pulmonary infiltrates
3. pulmonary infiltrates/fibrosis (no nodes) |
|
|
Term
| what are some dermatologic signs of sarcoidosis? |
|
Definition
| lupus pernio, e. nodosum on the shins |
|
|
Term
|
Definition
cytotoxic agents (e.g MTX)
antimicrobial agents (e.g chloroquin)
cytokine modulators (e.g infliximab) |
|
|
Term
| What is Microscopic Polyangiitis/Periarteritis? |
|
Definition
| Small vessel involvement resulting in rapidly progressive glomerulonephritis (RPGN) and pulmonary alveolar capillaries. The heart is also affected |
|
|
Term
| What is a classic finding in pts with Microscopic Polyangiitis/Periarteritis? What is the MC AB seen in this condition? |
|
Definition
Palpable purpura.
+ p ANCA titers |
|
|
Term
| What is Churg-Strauss Syndrome (Allergic Angiitis and Granulmatosis)? What type of pts are most commonly affected? |
|
Definition
A medium/small vessel vasculitis in pts with history of asthma/allergic rhinitis and peripheral eosinophilia.
Males btwn 15-70 yo are more commonly affected |
|
|
Term
| What are the clinical Sx of Churg-Strauss? How is it Dx? Tx? |
|
Definition
Clinical Sx: Atopic, asthmatic, skin and lung nodules (non-cavitary), peripheral neuropathy, eosinphilia
Dx: Eosinophils on CBC, High IgE, tissure bx reveals eosinophilic necrotizing granulomas.
Tx: Systemic steriods, Immunosuppressive agents |
|
|
Term
| What is Polyarteritis Nodosa (PAN)? |
|
Definition
A medium-sized, fatal vasculitis that affects any organ but skin, nerves, joints, GI, Kidneys are MC.
LUNGS are usually spared and most commonly occurs in MEN. |
|
|
Term
| What conditions are usually associated with PAN? |
|
Definition
|
|
Term
| What are the characteristics of PAN? How do most cases present? |
|
Definition
Characteristics: Painful skin ulcerations and extremity gangrene, bowel infarction, hepatic infarction or intra-hepatic bleed, renal involvement (ARF and HTN)
Presents as Mononeuritis multiplex (one peripheral nerve is invaded and damaged causing pain/parasthesias followed by motor deficit of same peripheral nerve) |
|
|
Term
| What is the most common nerve affected in assoc with PAN? |
|
Definition
| Sural nerve (around ankle)- Pts usually present with foot drop. |
|
|
Term
| What cutaneous sx are assoc with PAN? |
|
Definition
| Livedo reticularis, nodules, splinter hemorrhages, palpable purpura |
|
|
Term
| What neurologic sx are assoc with PAN? |
|
Definition
| Mononeuropathy (sural nerve/foot drop), Diffuse polyneuropathy, CVA |
|
|
Term
| What Renal/GI sx are assoc with PAN? |
|
Definition
| Segmental necrotizing GN, ischemic bowl and elevated LFTs |
|
|
Term
| What are the dx criteria for PAN? |
|
Definition
• Weight loss of > 4 kg since beginning of illness
• Livedo reticularis
• Testicular pain or tenderness
• Myalgias, weakness, or leg tenderness
• Mononeuropathy or polyneuropathy
• Development of hypertension
• Elevated BUN or creatinine unrelated to dehydration or obstruction
• Presence of hepatitis B surface antigen or antibody in serum
• Arteriogram demonstrating aneurysms or occlusions of the visceral arteries
• Biopsy of small or medium-sized artery containing granulocytes
• NOTE: at least 3/10 for PAN classification. |
|
|
Term
| What labs will help dx Classic PAN? |
|
Definition
Labs:
1. Elevated ESR/CRP
2. Leukocytosis
3. Screen for Hep B, C and HIV |
|
|
Term
| Where is bx performed in pts suspected to have PAN? What does the bx show? |
|
Definition
Skin and sural nerve are the most accessible.
Bx- Shows an acute inflammatory infiltrate with polymorphonuclear leukocytes and fibroid necrosis. |
|
|
Term
| What does an angiogram show in pts suspected to have PAN? |
|
Definition
| Multiple aneurysms (esp at buifurcations) and tapered narrowing with skip areas/beading |
|
|
Term
| What is the tx for class PAN? |
|
Definition
Steroids (IV pulse high doses)
IV Cyclophoshamide
*Survival is NOT high |
|
|
Term
| What is Giant Cell Arteritis? |
|
Definition
| A large vessel vasculitis that mostly affects the temporal artery and is considered a system dz (not isolated to temporal artery) |
|
|
Term
| What dz is Giant cell arteritis assoc with? |
|
Definition
|
|
Term
| What are the sx of Giant Cell Arteritis? |
|
Definition
| Unilateral sx common- H/A, scalp tenderness, jaw claudication, monocular vision loss (Amarosis fugax) and Aortic arch syndrome |
|
|
Term
| How is Giant Cell Arteritis Dx? What is the definitive dx? |
|
Definition
• Temporal artery tenderness or thickening
• Ischemic optic atrophy
• ESR >50, usually > 100
• CRP elevated
• Elevated LFTs in 30%
• Definitive DX: Abnl temporal artery bx with multi-nucleated giant cells and lymphocytic granulomas. |
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Term
| What is the tx of Giant Cell Arteritis? |
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Definition
• Usually requires high dose steroids for at least one month.
• RULE: Tx 1st with steroids to avoid visual loss, then bx w/in one week before histology affected by steroids.
• May require immunosuppressive therapy such as MTX, Azathioprine
• Usually resolves in 6-24 months |
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Term
| What is Wegner's Granulomatosis? |
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Definition
| A medium and small vessel vasculitis in young to middle aged adults. |
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Term
| What is the tx of Wegener's Granulomatosis? |
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Definition
• Steriods
• Cyclophosphamide
• Methotrexate
• ? TNF inhibitors
• ? Sulfa AB (Bactrim) |
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Term
| What is the classic triad for pts with Wegener's Granulmatosis? |
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Definition
URI, Pulmonary and renal involvement.
URI-Sinusitis, nasal and oral ulcerations-saddle-nose deformity
Pulmonary- SOB, hemoptysis, cavitating lesions, tracheal lesions
Renal- Proteinuria, hematuria, RBC casts, focal segmental GN on bx |
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Term
| What is myositis (idiopathic inflammatory myopathy/polymyopathy)? |
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Definition
| A symmetrical, proximal, painless muscle weakness that is common in 5-10 yo and 40-50 yo |
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Term
| What are the sx of myositis? |
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Definition
Dysphagia, neck muscles (flexors) are weak, pulm/CV sx, periorbital edema and cutaneous sx
*Pts have a hard time climbing stairs and doing other activities involving muscle fllexors |
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Term
| What are the cutaneous sx of myositis? |
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Definition
| Heliotrope rash, gottron's papules, sun-sensitive rash |
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Term
| What are the dx criteria for myositis? |
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Definition
• 1.Symmetrical weakness of limb girdle muscles and anterior neck flexors w or w/o dysphagia
• 2.Elevations in CPK
• 3.EMG c/w myopathy
• 4.Muscle biopsy + |
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Term
| How is the Definitive Dx for myositis? |
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Definition
Definitive Dx is a muscle bx showing:
– Perivascular infiltrate of inflammatory cells (lymphs)
– R/O on muscle bx: inclusion body myositis (vacuoles)
– Mitochondrial (ragged red fibers) |
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Term
| What is the tx for myositis? |
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Definition
• Steroids
• Methotrexate
• Azathhioprine
• Leflunomide
• Cyclophosphamide
• IVIG |
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Term
| Antiphospholipid Antibody Syndrome is assoc with which type of immunoglobulins? |
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Definition
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Term
| What is seen on EMG with myositis? Muscle enzymes? MRI? |
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Definition
EMG:
– Increased insertional activity
– Fibrillations
– Polyphasic motor unit potentials of low amplitude
Muscle Enzymes: elevated CPK, serum aldolase, SGOT
MRI- Increase in signal intensity to involved muscles (ie quads) |
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