Term
| What are the 4 early clinical signs of Scleroderma? |
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Definition
| Skin thickening on distal fingers, Arthralgias (pain in joints), Edematous phase (swelling of hands & wrists), Raynauds |
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Term
| Why is Raynauds a warning-sign in Scleroderma? |
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Definition
| Because after Raynauds can affect internal organs |
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Term
| What are the cutaneous signs of scleroderma? |
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Definition
| Abnormal/loss of capillaries under microscopy of nailfold, Raynaud's, Skin thickening |
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Term
| What are the 3 GI symptoms of scleroderma? |
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Definition
| Reflex esophagitis, Malabsorption, Pseudo-Obstruction caused by dysmotility |
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Term
| What causes the reflux esophagitis seen w scleroderma? |
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Definition
| Decreased lower esophageal sphincter function. |
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Term
| What are the pulmonary signs of scleroderma? |
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Definition
| Interstitial lung disease, pulmonary hypertension |
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Term
| 5 cardiac signs of scleroderma? |
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Definition
| Patchy myocardial fibrosis, ischemic cardiomyopathy, arrhythmias, pericarditis, myocarditis |
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Term
| What does glandular fibrosis from scleroderma lead to? |
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Definition
|
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Term
| What makes the CREST variant of scleroderma different? |
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Definition
| Generally more benign, less likely to have renal involvement |
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|
Term
| What does CREST stand for? |
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Definition
| Calcinosis, Raynauds, Esophageal dysmotility, Sclerodactyl, Telangiectasia |
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Term
| Which variant of scleroderma causes extremity pain & swelling after exertion? |
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Definition
|
|
Term
| Which variant of scleroderma is predominant in males? |
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Definition
|
|
Term
| High levels of what cause Eosinophilic Fasciitis? |
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Definition
|
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Term
| Linear Scleroderma, Morphea, & Sclerodema are all variations of what? |
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Definition
|
|
Term
| How does Scleroderma Renal Crisis present? |
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Definition
| Sudden onset of hypertension |
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Term
| What usually precedes Scleroderma Renal Crisis & why? |
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Definition
| Cold weather, intravascular volume depletion, steroid use; because these increase vasospasm & increase renin |
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Term
| How is Scleroderma Renal Crisis treated? |
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Definition
|
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Term
| What is the Graft vs Host theory of Scleroderma pathogenesis? |
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Definition
| Fetal anti-maternal GVH, persistent fetal cells leading to microchimerism, fetal cells see maternal cells as foreign & vice versa |
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Term
| Why is D-penicillamine given in scleroderma? |
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Definition
|
|
Term
| Usual age of Scleroderma onset? |
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Definition
|
|
Term
| Which serology test is specific for Scleroderma? |
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Definition
|
|
Term
| Which scleroderma variant is seen only in patients w chronic renal insufficiency? |
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Definition
| Nephrogenic Systemic Fibrosis |
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Term
| Nephrogenic Systemic Fibrosis is assoc w exposure to what? |
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Definition
|
|
Term
| What is used to treat the GI symptoms of scleroderma? |
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Definition
|
|
Term
| "String of Pearls sign" in barium enema indicates what? |
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Definition
|
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Term
| What age, gender, race is most affected by SLE? |
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Definition
|
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Term
| Combination of what 3 factors are implicated in SLE etiology? |
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Definition
| Genetic, Environmental, Hormonal |
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Term
| Abnormal apoptosis is a pathologic indicator of which disease? |
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Definition
|
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Term
| What is the mnemonic for clinical manifestation of SLE and what does it stand for? |
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Definition
| I'M DAMN SHARP - Immunoglobulins disorder, Malar rash, Discoid rash, ANA test +, Mucositis, Neurologic disorders, Serositis, Hematologic disorders, Arthritis (Jaccoud's), Renal disorders, Photosensitivity |
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Term
| In which disease is Jaccoud's arthritis found? |
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Definition
|
|
Term
| How is the mucosa effected in SLE? |
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Definition
|
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Term
| Describe the Discoid rash seen in scleroderma. |
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Definition
| Scaly, red patches of skin that cause scarring. |
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|
Term
| How does Jaccoud's arthritis present? |
|
Definition
| Small joints of hand/wrist, symmetric |
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|
Term
| What would the synovial fluid in Jaccoud's arthritis show? |
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Definition
|
|
Term
| How is the arthritis in SLE different from RA? |
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Definition
| Not erosive, inflammatory, or destructive to joints |
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Term
| What is the renal histological hallmark for SLE? |
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Definition
| "Wire loops" because of immune complex deposition in the glomerular basement membrane |
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|
Term
| What is the least severe class of lupus nephritis? |
|
Definition
| Class II - Mesangial proliferative LN |
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Term
|
Definition
|
|
Term
| What are the 5 classes of LN based on increased severity? |
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Definition
| Mesangial proliferative, Focal proliferative, Diffuse proliferative, Membranous, Advanced sclerotic |
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|
Term
| At which stage of LN is the glomerulus obliterated? |
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Definition
|
|
Term
| What is the accepted standard of diagnosis for SLE? |
|
Definition
| 3 multisystem symptoms & +ANA |
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Term
| For which symptoms of SLE would one give anti-malarials? |
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Definition
| Cutaneous, musculoskeletal, constitutional |
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Term
| What is the treatment for SLE assoc renal disease, organ disease, &/or life-threatening disease? |
|
Definition
| High dose steroids & immunosuppresants |
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Term
| Which symptom of SLE does high-intensity anticoagulation treat? |
|
Definition
| Antiphospholipid Syndrome |
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|
Term
| What type of endocarditis is assoc w SLE? |
|
Definition
| Libman-Sacks (non-bacterial) endocarditis |
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|
Term
| Which organs do vascultis of small vessels involve? Large vessels? |
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Definition
| Small vessels- skin lesions; Large vessels- ischemic disease in end organs |
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|
Term
| How are the skin & internal organs effected in small vessel vasculitis? |
|
Definition
| Skin lesions are universal, limited internal organ involvement |
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|
Term
| What is the most obvious clinical presentation of small vessel vasculitis? |
|
Definition
| Skin lesions called "Palpable Pupura" |
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|
Term
| A skin biopsy of small vessel vasculitis will show what? |
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Definition
| Leukocytic classic vasculitis |
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Term
| Infectious, Henoch-Schoenlein Purpura, Bechet's, & Hypersensitivity are the 4 types of what disorder? |
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Definition
|
|
Term
| What is the difference between the child & adult versions of Henoch-Schoenlein Purpura? |
|
Definition
| Mostly seen in kids, is self limited; Adults - purpura, polyarthritis, GI symptoms |
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Term
| IgA found in skin biopsy is the defining feature of which small vessel vasculitis? |
|
Definition
| Henoch-Schoenlein Purpura |
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|
Term
| Which vasculitis is caused by GC, meningococcus, sub-acute bacterial endocarditis, Rocky Mt spotted fever, or Hep C? |
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Definition
|
|
Term
| What is the difference btwn the ulcers in SLE & those in Bechet's small vessel vasculitis? |
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Definition
| SLE - painless; Bechet's - recurrent & very painful ulcerations @ mouth & genitals |
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Term
| What is Apthous Stomatitis & in which vasculitis is it seen in? |
|
Definition
| Recurrent, v painful mucosal ulceration of mouth & genitals; Bechet's small vessel vasculitis |
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Term
| What are the 2 more severe symptoms of Bechet's small vessel vasculitis seen in the MidEast & East? |
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Definition
| Blindness due to anterior uveitis. Phlebitis (vein inflammation) |
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Term
| What is the postulated etiology of Medium Vessel Vasculitis? |
|
Definition
| Deposits of immune system complexes in vessel wall--> inflammatory cascade w PMNs. |
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Term
| What are the 5 types of medium vessel vasculitis? |
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Definition
| Infectiouns, Buerger's, Polyarteritis Nodosa, Wegner's Granulomatosis, Churg-Strauss Vasculitis |
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|
Term
| Which type of vasculitis is seen in heavy smokers and causes no arthritis or systemic features? |
|
Definition
| Buerger's medium vessel vasculitis |
|
|
Term
| What are the 3 types of Systemic Necrotizing Vasculitis? |
|
Definition
| Polyarteritis Nodosa, Granulomatous Vasculitis, Wegeners Granulomatosis (PAN, GV, WG) |
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|
Term
| Which is the non-granulomatous medium vessel vasculitis? |
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Definition
|
|
Term
| Which vessels are spared in Polyarteritis Nodosa? |
|
Definition
|
|
Term
| Where are the focal lesions found in PAN? |
|
Definition
|
|
Term
| What clinical sign is pathomneumonic for PAN? |
|
Definition
|
|
Term
| In which organ do WG & CSV concentrate their disease? |
|
Definition
|
|
Term
| What is "saddle nose" and when is it seen? |
|
Definition
| Funny shaped nose due to chronic tissue destruction; WG & CSV |
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|
Term
| What is Wegener's Granulomatis also known as? |
|
Definition
| ANCA Associated Vasculitis |
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|
Term
| How is Churg-Strauss Vasculitis distinguished from Wegener's Granulomatosis? |
|
Definition
| Long history of asthma/allergies + hypereosinophilia in blood |
|
|
Term
| What 2 tests Dx Systemic Necrotizing Vasculitis? |
|
Definition
| Renal biopsy & angiography |
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|
Term
| What will be seen in a renal biopsy of a Systemic Necrotizing Vasculitis? |
|
Definition
| Immune crescenic glomerulonephritis |
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|
Term
| What will be seen via angiography in Systemic Necrotizing Vasculitis? |
|
Definition
|
|
Term
| What are you looking for in a biopsy of the affected artery in PAN? |
|
Definition
|
|
Term
| What will be seen in a biopsy of the affected organs in WG & CSV? |
|
Definition
|
|
Term
| In which vasculitis might you see a cavitating nodule in lung or an upper lung collapse? |
|
Definition
|
|
Term
| What causes the major organ symptoms seen in Large Vessel Vasculitis? |
|
Definition
|
|
Term
| What are the 2 types of Large Vessel Vasculitis? |
|
Definition
| Giant Cell Arteritis & Takayasu's |
|
|
Term
| Which vasculitis causes temporal artery inflammation which causes a headache? |
|
Definition
|
|
Term
| How is Giant Cell Arteritis Dx'd? |
|
Definition
| Temporal Artery biopsy - will show internal lamina destruction & granuloma into lumen |
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|
Term
| How is Giant Cell Arteritis treated? |
|
Definition
|
|
Term
| At what age are people more likely to get Takayasu's? |
|
Definition
|
|
Term
| When does the damage to main organs from ischemia happen in Takayasu's? |
|
Definition
| When symptoms are subclinical |
|
|
Term
| What test is done to Dx Takayasu's? |
|
Definition
|
|
Term
| What are the treatment options for Takayasu's? |
|
Definition
| Steroids, immunosuppressants, vascular reconstruction |
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