CT Disorders and Vasculitides Flashcards
What is used to test for autoimmune disease?
What feature of this test makes it important to gather more information about the patient?
1) ANA
2) Not specific
Systemic lupus Erythematosus has autoantibodies to what antigens?
What type of hypersensitivity is it?
1) Nuclear
2) Type III
What are notable cutaneous features of SLE?
1) Malar butterfly rash
2) Photosensitivity
What is a notable hematologic feature of SLE?
Thrombosis
What are notable cardiopulmonary features of SLE?
1) Pericarditis
2) Libman-Sacks endocarditis
3) MI
What body position does pericarditis get better/worse?
What does it look like on ECG?
What can be heard on auscultation?
1) Worse in supine, better with sitting upright
2) ST elevation
3) Pericardial friction rub
Why are SLE patients at an increased risk of MI?
Accelerated atherosclerosis
What is a notable renal feature of SLE?
Nephritis
Where does the malar butterfly rash spare on the face?
Nasolabial folds
What specific antigens do SLE patients have autoantibodies for?
Which one correlates with disease activity?
1) dsDNA (Correlates with disease activity)
2) Sm (Smith)
What complement proteins will be decreased on SLE serology because of their increased consumption with this condition?
C3 or C4
What causes Libman-Sacks endocarditis in SLE?
Immune deposits on the valves
What are the treatment/prevention options for SLE?
1) Avoid sun exposure/wear sunscreen
2) NSAIDs
3) Glucocorticosteroids
4) Hydroxychloroquine
In the early years after diagnosis what are causes of SLE mortality?
In later years?
1) Opportunistic infections, kidney, or CNS disease
2) MI or thromboembolic events
What does type one antiphospholipid antibodies cause biologic false-positive tests for?
Syphilis
What is type two antiphospholipid antibodies?
What does it cause risk factors for?
1) Lupus anticoagulant
2) Venous/arterial thrombosis and miscarriage
What is type three antiphospholipid antibodies?
The antibodies are directed towards what serum cofactor?
1) Anti-cardiolipin antibodies
2) Beta-2 glycoprotein I
What is the treatment for APS?
Anticoagulation
Both SLE/APS cause retinopathy, what diagnostic feature is seen in the eye?
Cotton wool spots
What symptoms are not seen in Lupus-like syndrome/Drug-induced?
No renal or neuro symptoms
Lupus-like syndrome/Drug-induced are positive for what antibodies?
Anti-histone Abs
What may happen to SLE patients when given Sulfa antibiotics?
Exacerbate lupus symptoms
Neonatal lupus affects children born of mothers with what Abs?
What can this condition cause?
1) Anti-Ro (SSA) or La (SSB) Abs
2) Permanent complete heart block
Discoid lupus erythematosus (DLE) is a variant of which lupus?
Where does it most commonly occur?
What is it characterized by?
1) Cutaneous lupus
2) Head
3) Inflammatory plaques that evolve into atrophy, disfiguring scars
What is the hallmark of scleroderma?
Thickening and hardening of the skin and visceral organs (fibrosis)
What causes the dry itchy skin seen in scleroderma?
Obliteration of eccrine sweat and sebaceous glands
What is usually the first symptom seen in caucasian scleroderma patients?
In African American patients?
1) Secondary Raynaud phenomenon
2) Changes in skin pigmentation
Which type of scleroderma is seen in children and causes discrete areas of discolored skin induration?
Localized scleroderma
How does raynaud’s present in localized scleroderma?
What are the patches seen on the skin called?
1) There is no Raynaud’s
2) Morphea
Which type of scleroderma is associated with the CREST syndrome?
Limited Cutaneous Systemic Sclerosis
What is the CREST syndrome?
1) Cutaneous calcinosis
2) Raynaud’s (2⁰)
3) Esophageal dysmotility
4) Sclerodactyly
5) Telangiectasia
How are the vascular manifestations more pronounced in limited cutaneous systemic sclerosis?
1) Digital ischemia
2) Pulmonary artery HTN
How does pulmonary artery HTN present in limited cutaneous systemic sclerosis?
How does esophageal dysmotility present?
1) As shortness of breath
2) GERD
Which type of scleroderma causes fibrosis, loss of body hair, and impaired sweating?
Diffuse cutaneous systemic sclerosis
In what ways do diffuse cutaneous systemic sclerosis involve internal organs?
1) Renal crisis
2) Interstitial lung disease
Which type of scleroderma causes interstitial lung disease?
Which causes pulmonary artery HTN?
1) Diffuse
2) Limited
What does interstitial lung disease sound like on auscultation?
How is it diagnosed?
1) Fine “velcro” crackles (rales)
2) Pulmonary function test
How is pulmonary artery HTN diagnosed?
What does PAH increase the incidence of?
1) Right heart catheterization
2) Bronchoalveolar carcinoma
Renal crisis due to diffuse cutaneous systemic sclerosis can cause abrupt onset of?
1) Malignant hypertension
2) Hemolytic anemia
3) Progressive renal insufficiency
High doses of what treatment should be avoided in diffuse scleroderma?
Why?
Glucocorticoids because they induce renal crisis
What GI manifestations are seen with scleroderma?
1) Barrett esophagus
2) Gastric antral vascular ectasia (GAVE Syndrome) aka watermelon stomach
3) Primary biliary cirrhosis
What does Barrett esophagus increase the risk for?
Esophageal adenocarcinoma
What effect does scleroderma have on msk?
On the thyroid?
1) Carpal tunnel
2) Hypothyroid
What is found on serology for diffuse scleroderma?
1) Anti-Scl 70 aka Anti-(DNA) topoisomerase I
2) Anti-RNA polymerase III
What is found on serology for limited scleroderma?
Anti-centromere
What are the Sicca symptoms seen in Sjӧgren syndrome?
1) Dry eyes, mouth, vagina
2) Parotid enlargement
Xerostomia in Sjӧgren patients can lead to what complications?
1) Candida
2) Dental caries
Keratoconjunctivitis sicca is due to?
How is it diagnosed?
1) Inadequate tear production
2) Schirmer test
What does Sjӧgren syndrome have a strong association with?
B cell non-Hodgkin lymphoma (MALT lymphoma)
What is found on serology for Sjӧgren syndrome?
1) Polyclonal Hypergammaglobulinemia
2) Anti SSA/Ro
3) Anti SSB/La
What is essential for diagnosing Sjӧgren syndrome?
Lip biopsy
What treatments should Sjӧgren syndrome patients avoid?
Atropinic drugs and decongestants
What causes symmetrical proximal muscle weakness making it difficulty rising from a chair or bathtub?
Inflammtory myopathies
What enzymes do we see elevated in serum with inflammatory myopathies?
Creatine kinase and aldolase
What causes proximal muscle weakness early with distal muscle weakness in later disease course while having no sensory symptoms?
Dermatomyositis
What are the characteristic skin lesions seen with dermatomyositis?
1) Gottron’s patches/papules
2) Heliotrope rash
3) Periungual and V-neck (poikiloderma shawl sign) erythema
What is heliotrope rash?
Periorbital edema and purplish suffusion over eyelids
What does dermatomyositis increase the risk for?
Occult malignancy
What is found on serology for dermatomyositis and polymyositis?
What is the main treatment for dermatomyositis and polymyositis?
1) Anti Jo-1
2) Glucocorticoids
While polymyositis has many similarities to dermatomyositis what seperates it?
No skin changes
What confirms diagnosis of polymyositis on biopsy?
Endomysial inflammation
Unlike most autoimmune diseases that tend to affect females more, which ones are more prevalent in males?
1) Inclusion body myositis
2) Granulomatosis with Polyangiitis (GPA) aka Wegener’s Granulomatosis
3) Polyarteritis Nodosa
4) Kawasaki Disease
5) Thromboangiitis Obliterans
What causes weakness in finger flexion or quadriceps weakness?
Inclusion body myositis
What confirms diagnosis of inclusion body myositis on biopsy?
What labs do we find for IBM?
What serology?
1) Rimmed vacuoles
2) Mild elevation or normal CK
3) Anti-cN1A autoAbs
Henoch-Schönlein Purpura (HSP) affects what sized vessels?
What population is it more common in?
1) Small vessel
2) Kids
What is the most common symptom seen with Henoch-Schönlein Purpura?
How is it diagnosed on biopsy?
What is the Tx?
1) Palpable purpura without thrombocytopenia
2) IgA deposits
3) Glucocorticoids
Goodpasture syndrome affects what sized vessels?
What is the cause of death if not treated?
How is it diagnosed on renal biopsy?
How is it treated?
1) Small vessel
2) Pulmonary hemorrhage
3) Deposition of anti-basement membrane antibodies
4) Plasmapheresis
Granulomatosis with Polyangiitis (GPA) aka Wegener’s Granulomatosis affects what sized vessels?
How is it diagnosed on biopsy?
On serology?
1) Small vessel
2) Granulomas
3) ANCA
What are the hallmarks of GPA?
1) Granulomatous inflammation
2) Necrotizing vasculitis
3) Segmental glomerulonephritis
Because of the renal involvement of GPA what may we see?
How does it affect the respiratory tract?
1) Hematuria and proteinuria
2) Nasal involvement usually saddle nose
Eosinophilic Granulomatosis with Polyangiitis (EGPA) aka Churg-Strauss Syndrome involves what sized vessels?
What area of the body does it mostly affect?
What are the hallmarks?
1) Small and medium
2) Respiratory tract
3) Asthma and Eosinophilia leading to vasculitis with granulomas
How is EGPA diagnosed on CBC?
On Serology?
On lung biopsy?
1) High eosinophil count
2) ANCA
3) Granulomas
When Behcet Syndrome affects the large vessels what does it cause?
When there is venous involvement?
When it affects small vessels what does it cause?
What is found on serology?
1) Aneurysms
2) DVT
3) Recurrent mouth ulcers, genital ulcers, and eye inflammation (uveitis)
4) HLA-B51
Thromboangiitis Obliterans aka Buerger Disease affects what sized vessels?
What population does it only occur in?
How is it diagnosed on angiography?
What is the treatment?
1) Medium
2) Smokers
3) Corkscrew appearance
4) Stop smoking
Polyarteritis Nodosa affects what sized vessels?
What virus is it associated with?
What skin symptom is common to see?
Majority of patients have what peripheral nerve issue?
What renal issue?
What respiratory issue?
1) Medium
2) HBV virus
3) Digital gangrene
4) Foot drop
5) Renin mediated HTN
6) None, lungs spared
How is diagnosis of Polyarteritis Nodosa confirmed by biopsy?
BY angiogram?
By serology?
1) Fibrinoid necrosis and NO granulomas
2) Microaneurysm
3) HBsAg, HBsAb, and HBeAg (Hep B)
Kawasaki Disease AKA Mucocutaneous lymph node syndrome affects what sized vessels?
What age population does it affect?
Where is incidence the highest?
What is the hallmark?
What is morbidity due to?
1) Medium
2) Less than 5yo
3) Japan
4) Strawberry tongue
5) Aneurysm or MI
How is Kawasaki disease treated?
1) IVIG within 10 days of sx
2) High dose aspirin
Takayasu Arteritis affects what sized vessels?
What vessel in particular?
What age population does it affect?
What is seen on angiography?
Why is it known as the pulseless disease?
1) Large
2) Aorta
3) Less than 40
4) Long smooth tapered stenosis
5) Obliterates UE peripheral pulses
What causes the HTN in Takayasu Arteritis?
What does the HTN lead to?
What aortic complications are seen?
1) Renal artery stenosis
2) Copper wiring retinopathy
3) Dilations, regurgitation, aneurysm, and rutgers
How is Takayasu Arteritis diagnosed on histology?
Granuloma with some giant cells
What biomarkers are elevated in Giant cell arteritis (GCA) and polymyalgia rheumatica (PMR)?
How are the two conditions related?
1) ESR and CRP
2) They often coexist
Giant Cell Arteritis (GCA) aka Temporal Arteritis affects what sized vessels?
Which vessels in particular?
What is the gold standard for biopsy?
What is seen on biopsy?
How is it treated?
What happens if there’s no treatment?
1) Large vessels
2) Cranial arteries and aortic arch
3) Temporal artery
4) Multinucleated giant cells
5) Glucocorticoids before biopsy
6) Blindness
What are hallmarks of GCA?
1) Jaw claudication
2) Headache
3) Visual abnormalities
Polymyalgia Rheumatica (PMR) is associated with what symptoms?
How is it diagnosed?
1) Stiffness, soreness, and pain in proximal muscles
2) Everything normal except elevated ESR and CRP
Which type of Raynaud is benign symmetric and is due to exaggerated physiologic response to cold?
Which one is unilateral is is more severe as it can lead to ischemia?
1) Primary
2) Secondary
What test is used to differentiate between primary and secondary raynaud?
Which is suspected if results show distortion with widened and irregular loops, dilated lumen and areas of vascular “dropout.”?
1) Nailfold capillaroscopy
2) Secondary
