McGowan Vasculitidies Flashcards
Etiology of SLE?
- Multisystem inflammatory AI disorder
- Type III hypersensitivity
What can be seen on PE for a patient with SLE?
- Constitutional sx such as fever, fatigue, malaise, weight loss
- Cutaneous rashes and photosensivitity
- Arthritis
- Hematologic issues
- Pericarditis
- Libman-Sacks endocarditis
- Risk MI
- Nephritis
SLE diagnosis?
- Serolgy:
- dsDNA
- does correlate with disease activity
- Smith ab
- doesnt correlate with disease activity
- dsDNA
SLE treatement?
- Avoid sun exposure
- NSAIDs for pain (caution with renal dz)
- Glucocorticosteroids (systemic or topical)
- Hydroxychloroquine
Management and prevention of SLE?
- Minimize risk factors for atherosclerosis
- avoid or quit smoking
- Flu & pneumococcal vaccine
- Cancer screenings
What is the prognosis/COD in the early years after a diagnosis with SLE?
- Infections especially in those treated with immunosuppressives
- Active SLE COD is kidney or CNS disesase
What is the typical COD in later years after a diagnosis of SLE?
- Accelerated atherosclerosis linked to chronic inflammation
- MI is 5x more likely
- Thromboembolic events
What are the three primary autoantibodies seen in Antiphospholipid Ab syndrome?
- Anti-Cardiolipid antibodies aCL
- Lupus anticoagulant
- Beta 2 glycoprotein I
A 27 year old female patient comes in to the ED. She is having a miscarriage, this is her 3rd one over the past 3 years. Her PMH is insignificant except for a DVT after a 15 hour plane ride. She takes no medications. What are you suspecting?
Antiphospholipid Ab Syndrome
What Ab causes false positives for syphilis?
aCL, seen in APS
What Ab prolongs PTT and is not corrected by adding normal platelet free plasma, and what is it seen in?
Lupus Anticoagulant (LA) seen in APS
How do you treat APS?
Indefinite systemic anticoagulation
What two disorders can you see cotton wool spots in?
- SLE
- APS
How do you diagnose Drug induced lupus (lupus like syndrome)?
- +ANA
- + Anti histone abs
Hx/PE of Dil?
- No renal or neuro sx but looks similar to SLE
- Medications such as:
- Procainamide
- Hydralazine
- Isoniazid
- Methyldopa
- Lithium
- Phenytoin
- Nitrofurantoin
- Sulfasalazine
- HCTZ
- Simvastatin
*List of meds is purple
Neonatal lupus affects children born of mothers with ___.
Neonatal lupus affects children born of mothers with Anti Ro (SSA) abs.
How will a baby with neonatal lupus present?
- Congenital heart block
- Transient rashes, thrombocytopenia, hemolytic anemia, arthritis
How do you diagnose neonatal lupus
- + anti ro in mom
- Serial echocardiograms and obstetric sonograms
Treatment and management of neontal lupus?
- Delivery if fetus is in distress
- Dexamethasone tx of mom with in utero heart block sometimes prevents progression to complete heart block
- Good outcome if managed
Discoid lupus etiology and presentation?
- Independent or manifestations of SLE most common on the head
- Well defined inflammatory plaques that evolve into atrophic disfiguring scars
Diagnosis and treatment of Discoid lupus?
- Biopsy
- Photo protection and topical anti inflammatory agents or systemic antimalarial drugs
Hallmark of SSc? (scleroderma)
- Thickening of the skin
What is Localized Scleroderma? How does it present and how do you diagnose and treat?
- Not systemic but can involve joints or muscles in the area
- Discrete patches of discolored skin induration, usually asx (Morphea)
- NO raynauds
- diagnose with Hx and biopsy (indistinguishable from other SSc)
- PT, Steroids, phototherapy
How does Limited Scleroderma present? (CREST syndrome)
- Calcinosis
- Raynauds
- Esophageal dysmotility
- Sclerodactyly
- Telangiectasia
- Progressive Pulmonary artery HTN presenting as SOB
How do you diagnose CREST? (LCSSc)
- Dx made in advanced cases
- + anti centromere ab
- +ANA
- PE, XR, UA, BX
How do you treat LCSSc?
- No therapy alters disease course
- Education
- Ca channel blockers for raynauds
- ACE inhibitors for htn and limiting renal disease
- PPI reffulx
- Glucocorticoids (high doses can lead to development of renal crisis)
Hx and PE of Diffuse Scleroderma?
- Interstitial lung disease
- Renal crisis
- skin induration and hyper/hypo pigmentation leading to loss of body hair and impaired sweating
- Fibrotic joints
When comparing CREST(LCSSc), Localized scleroderma (SSc) , and diffuse scleroderma, which syndrome has Raynauds occuring early and which one has internal organ involvent?
- Raynaud’s occurs early in CREST, later in diffuse and, not at all in Localized
- SSc does not involve internal organs, LCSSc involves some, DCSSc involves internal organs
Your patient has ___ and you hear dry course velcro like crackles on lung auscultation.
Your patient has Diffuse Scleroderma and you hear dry course velcro like crackles on lung auscultation.
How do you diagnose DCSSc?
- Anti Scl 70 aka Anti Topoisomerase I
- Anti RNA pol III
- ANA
- CXR or CT of chest
- PFTs
What is the primary cause of morbidity and mortality in systemic sclerosis?
- Pulmonary involvement
- PAH and ILD
Describe the effects of systemic sclerosis on the renal system.
- CKD
- proteinuria
- elevation creatine
- Htn
- Renal crisis is abrupt onset of:
- malignant hypertension
- hemolytic anemia
- Progressive renal insufficiency
- more common in diffuse SSc
Describe Pe findings for a patient with Sjogren syndrome
- Sicca symptoms
- dry eyes mouth vagina tracheo bronchial
- Keratoconjunctivitis sicca: foreign body sensation due to inadequate tears
- Parotid gland enlargement
- associated with malt lymphoma
How do you diagnose Sjogren?
- Polyclonal hypergammaglobulinemia
- + anti SSA/Ro
- + Anti SSB/La
- Labial salivary gland lip biopsy
How do patients with Dermatomyositis present>? (DM)
- Weakness without sensory sxs
- Ages 7-15 and 30-60
-
Skin lesions
- gottrons patches
- Helitrope lash
- V neck erythema
- Poikiloderma “Shawl Sign”
What should you look for when you diagnose a patient with Dermatomyositis?
Increased risk of occult malignancy
How do you diagnose DM?
- Elevated CK and adolase
- Serology Anti Jo-1
- Bx shows perimysial and perivascular inflammation perifascicular atrophy
How do you treat Dm?
Glucocorticoids
How does Polymyositis present?
- NO skin changes as sseen in DM
- Subacute proximal muscle weakness over weeks to months (same as DM)
- 30-50+ yo
Labs for PM?
Similar to DM (bx different)
- Elevated CK and adolase
- Anti Jo-1
- Endomysial inflammation with invasion of non necrotic muscle fibers without features suggestive of another dx
Inclusion body myositis presnetation and treatment?
- Males>Females
- Weakness in finger flexion or quads
- Refractory to tx so its supportive
How do you diagnose IBM?
- Mild elevation or normal CK
- anti cN1A autoabs
- Bx shows Endomysial inflammation rimmed vacuoles invasion of non necrotic muscle fibers
What are the vasculitides that impact small vessels?
- IgAV aka Henoch Schonlein Purpura (HSP)
- Anti-GMP (Goodpasture)
- Granulomatosis with Polyangiitis (GPA)
What are the vasculitides that impact medium vessels
- Thromboangiitis Obliterans (Beurger Disease)
- Kawasaki
- Polyarteritis Nodosa (PN)
What are the vasculitides that impact Large vessels
- Takayasu Arteritis
- Giant cell arteritis (temporal arteritis)
Who does HSP impact and what is the tetrad?
- Kids
- Palpable purpura without thrombocytopenia
- Arthritis
- Ab pain
- Renal disease
IGA vasculitis (HSP) biopsy results and tx?
- Bx shows IgA deposits
- Tx is supportive and glucocorticoids
Goodpasture syndrome presentation dx and treatment?
- Hematuria
- Hemoptysis
- Diagnose with renal bx showing deposition of anti basement membrane autoantibiodies
- Tx with plasmapheresis and glucocorticoids
Hallmarks of Granulomatosis with polyangiitis (aka Wegener’s granulomatosis)
- Granulomatous inflammation
- Necrotizing vasculitis
- Venous thrombolytic events
- Segmental glomerulonephritis
- URI/LRI
- *90% cases have nasal involvement
- Saddle nose
- Lungs show cavitary lesions
How do you diagnose and treat GPA?
- ANCA
- Bx shows vessel changes with granulomas
- NO smoking and give high dose glucocorticoids
Who does GPA effect more?
Males
What are the Hx/PE findings for Eosionophilic granulomatosis with polyangiitis (EGPA) aka Churg Strauss syndrome?
- Respiratory tract
- Asthma + Eosinophilia → vasculitis with granulomas
What are the phases to EGPA?
- Prodromal phase:
- allergic disease
- Eosinophilia tissue infiltration phase:
- High eosinophils in circulating blood
- Vasculitis phase:
- Systemic necrotizing and palpable purpura
Bechet syndrome?
- Large vessel aneurysms
- DVT
- Triad of small vessels:
- Recurrent mouth ulcers
- Genital ulcers
- Eye inflammation
What vasculitis do you see pathergy in? (pustules at site of sterile needle prick)
Bechet syndrome
How do you diagnose Bechet and treat?
- HLA B51
- Low dose glucocorticoid
Thromboangiitis Obliterans (Buerger disease) Hx/PE diagnostics and treatment?
- Young men <45
- ONLY occurs in those who smoke
- Loss of digits
- Angiography shows corckscrew appearance
- Tx is to stop smoking
Polyarteritis nodosa Hx and PE?
- Assoc with HBV
- Livedo reticularis, ulcers, digital gangrene
- 80% have vasculitis neuropathy and foot drop
- Newly acquired HTN
- lungs spared
How do you diagnose and treat polyarteritis nodosa?
- Biopsy shows fibrinoid necrosis and NO granulomas
- Angiogram shows micro aneurysm
- Serology ANCA and check HBV serology
- Tx with glucocorticoids
- Tx HBV if present
Kawasaki Disease Hx and PE? (mucocutaneous lymph node syndrome)
- Worldwide but highest rate in Japan, <5 yo (M>F)
- Fever lymphadenopathy rash strawberry tongue
What accounts for morbidity in Kawasaki?
Coronary aneurysm or MI
Tx for Kawasaki?
- IVIG w/n 10 days sx
- High dose ASA
Takayasu Arteritis hallmarks?
- Aorta is involved and can result in aneurysm, rupture, dilations and regurgitation
- Pulseless disease (obliterates UE peripheral pulses)
- 50% have pulmonary involvement
- Copper wiring retinopathy
- Renal artery stenosis
Overlapping features between GCA and PMR?
- Frequently co exist
- F>M
- >40-50
- Constitutioinal sx
- Elevated ESR and CRP
- Tx with glucocorticoids
Giant cell arteritis (aka Temporal arteritis) Hx and PE.
- Cranial arteries tender
- Dull headache
- Jaw claudication (painful chewing)
- Associated with Polymyalgia Rheumatica
- Amaurosis fugax or diplopia
How do you diagnose and treat GCA?
- Very high ESRS
- HLA DR4
-
Temporal artery bx is gold standard
- see multinucleated giant cells
- Start glucocorticoids immediately as this could lead to blindness
Hx and PE findings with Polymyalgia Rheumatica (PMR)?
- Stiffness, soreness and muslce pain
- Starts in morning and throughout the day
- Feels weak subjectively but not actually, it is due to pain
Diagnosis and treatment of PMR?
- Everything normal except ESR and CRP elevated
- no inflammation on muscle bx and muscle enzyme labs are normal
- EMG normal
- Tx with glucocorticoids
Differentiate primary from secondary Raynaud’s phenomenon.
Primary:
- Benign
- Symmetric
- Exaggerated physioilogic response to cold or emotion
- 15-30 yo F>M
- Normal Nailfold capillaroscopy
Secondary:
- >30 yo
- Unilateral
- Secondary to something
- More severe ischemia
- Nailfold capillaroscopy is distoreted with widened and irregular loops dilated lumen and areas of vascular dropout
