Connective Tissue Dx Flashcards by Stephan Green

What’s an ANA?

Anti-nuclear antibody test, loosely associated w/ underlying autoimmune dxs

Looks for antibodies that target self-antigens in the nucleus of cells

Labs use different methods to test for ANAs

How well did you know this?

Not at all

Perfectly

What does the ANA test homogenous staining pattern imply?

Ab directed against chromatin, histones, proteins, and DNA

Drug-induced SLE
Sjogren’s syndrome
SLE

How well did you know this?

Not at all

Perfectly

What does the ANA test speckled staining pattern imply?

Directed against Sm antigens, not as uniform as centromere

Mixed Connective tissue dx
Diffuse Systemic Sclerosis (Scleroderma)
Sjogren’s syndrome
SLE

How well did you know this?

Not at all

Perfectly

What does an ANA test centromere staining pattern imply?

Resting cells = dispersed evenly
Mitotic cells = localized to chromosomes

Limited Systemic Sclerosis (CREST)

How well did you know this?

Not at all

Perfectly

What does an ANA test nucleolar staining pattern imply?

Staining of nucleolus, Ab against RNA

Diffuse Systemic Sclerosis (Scleroderma)
SLE

How well did you know this?

Not at all

Perfectly

What criteria is used to support an SLE diagnosis?

Malar rash
Oral ulcers
Photosensitivity
Arthritis
Hematologic disorders
Positive ANA
Kidney dx (nephritis)
Neurologic dx (seizures, psychosis)

Cardiac valve dx
Reydaud’s phenomenon
Atherosclerosis
Pericarditis/pleurisy

How well did you know this?

Not at all

Perfectly

What is SLE? Type of hypersensitivity? Progression? Sex? Race? Environmental factors? Pt most likely to die from?

Autoimmune Inflammatory disorder, Ab to nuclear antigens

T3 hypersensitivity
remissions and relapses
F > M
AA & Hispanics > white, poor socioeconomic status
UV light, EBV
atherosclerosis or kidney dx

How well did you know this?

Not at all

Perfectly

What’s the serology findings for SLE?

ANA pos

anti-dsDNA = correlates w/ dx activity
Sm (Smith)

Dec complement (C3 &amp; C4) d/t consumption
- normalizes in remission

How well did you know this?

Not at all

Perfectly

What dx process is often associated w/ SLE? Explain + tx.

Antiphospholipid Antibody Syndrome (APS)

3 types of antibodies (one = false positive for syphilis, two = lupus anticoagulant, three = anti-cardiolipin > Beta2GP1)
tx: anticoagulation (Warfarin, Heparin/LMWH in pregnancy)

How well did you know this?

Not at all

Perfectly

Cotton wool spots on the retina may suggest what?

SLE/APS retiopathy

- many things cause cotton wool spots though

How well did you know this?

Not at all

Perfectly

What are the serology findings in Lupus-like syndrome/Drug-induced?

ANA pos

- anti-histone antibodies (homogenous stain pattern)

How well did you know this?

Not at all

Perfectly

What drug class can cause an SLE flare?

Sulfa antibiotics

How well did you know this?

Not at all

Perfectly

What antibodies convey neonatal lupus? What’s a symptom in the neonate?

Anti Ro (SSA) 
Anti La (SSB)

Permanent/complete heart block

How well did you know this?

Not at all

Perfectly

What’s the tx for SLE?

Avoid sun exposure
- wear sunscreen
NSAIDs
Corticosteroids
Hydroxychloroquine

How well did you know this?

Not at all

Perfectly

What’s a Type III Hypersensitivity response? What’s it associated w/?

Immune complex-mediated > complement activation > recruitment of leukocytes > release of cytokines and enzymes

SLE

How well did you know this?

Not at all

Perfectly

What is Discoid Lupus?

Chronic skin condition w/ red, inflamed, crusty/scaling patchy lesions

How well did you know this?

Not at all

Perfectly

What is Scleroderma (systemic sclerosis) generally? What are the three types?

Progressive dx involving the thickening/hardening of the skin w/ fibrosis of skin and organs
- obliterates eccrine glands > dry itchy skin

Diffuse (dcSSc), Limited (lcSSc), Localized

How well did you know this?

Not at all

Perfectly

Differentiate between the different variants of Scleroderma:

Diffuse: systemic/diffuse, extremities + trunk involved, early & progressive organ involvement, worst prognosis, Raynaud (later), Scleroderma Renal Crisis

Limited: face/neck and distal extremities, pulmonary HTN and Raynaud’s (early), CREST syndrome, indolent course w/ good prognosis

Localized: benign, affects children, no Raynaud’s or systemic sxs, presents as areas of discolored skin (patches = morphea, coalesced patches = generalized morphea)

How well did you know this?

Not at all

Perfectly

What does CREST in CREST syndrome stand for?

Study These Flashcards

Calcinosis cutis (calcium deposits in the skin)
Raynaud’s (secondary, fingers don’t receive proper blood supply in cold, under stress)
Esophageal dysmotility
Sclerodactyly (thickening and tightness of skin on fingers/toes)
Telangiectasia (blood vessel patterns seen on skin)

What’s the serology for Scleroderma?

Study These Flashcards

ANA pos

Diffuse: anti-Scl 70/topoisomerase I, anti-RNA polymerase III

Limited: anti-centromere

What GI manifestations can arise in Scleroderma?

Study These Flashcards

Malnutrition: fat, protein, B12, Vit D def
GERD > Barrett esophagus > inc adenoCA risk
Gastric antral vascular ectasia (GAVE, watermelon stomach)

What’s the primary cause of morbidity and mortality in Scleroderma?

Study These Flashcards

Pulmonary path

interstitial lung dx – diffuse
pulmonary artery hypertension (PAH) – limited

Regarding Scleroderma, which variant is CKD most likely to occur in? How might it present?

Study These Flashcards

Diffuse

Abrupt onset of HTN, hemolytic anemia, renal insufficiency

Sjogren syndrome is what? What CA does it have a strong association to?

Study These Flashcards

Immune-mediated destruction of the exocrine glands > sicca sxs

dry eyes/inadequate tear production
xerostomia > dental caries (cavities)
vaginal dryness
parotid gland enlargement

B-cell non-Hodgkin lymphoma

Sjogren Syndrome can be a secondary manifestation in certain dxs, of which are?

``` RA SLE Scleroderma Polymyositis Autoimmune thyroiditis Polyarteritis Interstitial pulmonary fibrosis Primary biliary cirrhosis/cholangitis ```

What's the serology or Sjogren's Syndrome?

ANA and RF pos Hypergammaglobulinemia Anti SSA/Ro pos & Anti SSB/La pos (come together always)

What is the state of ESR and CRP in inflammatory myopathies?

Normal

A pt (7-15, 30-60) presents with weakness to mm. They have periorbital edema with a purplish suffusion over the eyelids and a positive Shawl sign (erythema over neck/shoulders, upper chest and back). No sensory sxs noted. What does this pt have? What's the pt at risk for? What are common associated findings?

Dermatomyositis ``` Occult malignancy (ovarian mc, check CA-125) - get a transvaginal ultrasound ``` Perifascicular atrophy Elevated CK and Aldolase Anti-Jo1, Anti-Mi2, Anti-MDA5, anti-P155/P140

What are Gottron's patches/papules and what are they associated w/?

Raised lesions overlying the dorsa of DIP, PIP, and MCP joints Inflammatory myopathies

Pt (30-50+) presents w/ c/o m weakness. No skin changes noted on PE. Labs show an increased CK. What would labs and a biopsy show? What does this pt have?

Anti-Jo1, endomysial inflammation Polymyositis

How do you tx DM/PM?

Corticosteroids

A > 40-50 yo caucasian male presents w/ finger flexion and/or quad weakness. CK is slightly elevated.. close to normal. What's the dg? What's expected on the biopsy? What's the tx?

Inclusion body myositis (IBM) Rimmed vacuoles w/ anti-cN1A autoantibodies Supportive only (no tx available)

A < 40 yo Asian female presents with odd feeling in chest. Upon PE, her pulses are not found in the UE, but are in the LE. Fundoscopic exam shows "copper wiring" retinal infarctions. What dx is this and how is a diagnosis made? What's occurring? What are associated sxs? What's the tx?

Takayasu Arteritis ("pulseless dx"), dg made by MRI or CT Long smooth tapered stenosis of the aorta and its branches Pulmonary involvement, retinopathy, renal a stenosis, aortic dilations, aortic regurg, aneurysm, aortic rupture Glucocorticoids

What are some attributes of Henoch-Schonlein Purpura (HSP)?

Palpable purpura (but no thrombocytopenia) Common in kids IgA deposits Abd pain, arthritis, glomerulonephritis

A pt presents w/ Behcet Syndrome. Where is this often seen regionally? What's the genetic component? What's the triad of findings for this pt?

``` Silk route (Turkey, Asia, Mid East) HLA-B51 ``` Recurrent mouth ulcers, genital ulcers, uveitis (eye inflammation)

A male patient w/ HBV comes to your clinic c/o fever, malaise, wt loss, and peripheral neuropathy w/ foot drop. He's noticed nodules on his skin. Also some abd pain, and his BP is high on PE (which is says is abnormal). What does he have? What organ system has been spared? What's helpful in diagnostic work-up? What's the tx?

Polyarteritis Nodosa Lungs Infiltration and destruction of vasculature by inflammatory cells > necrosis - angiogram - ANCA negative Corticosteroids

A young Asian child < 5 yo presents w/ redness and swelling of their feet and hands. What other sxs would you see in this pt? What do they have? What's the tx? What might cause death here?

Swollen lymph nodes Strawberry tongue w/ dry cracked lips Fever Medium vessel involvement Kawasaki dx IVIG and high-dose ASA (yes, risking Reys syndrome) Aneurysms or MI (may occur years later)

A > 40 yo male presents w/ granulomatous inflammation of the respiratory tract (inside the nares). On x-ray, alveolar hemorrhages and cavitary lesions are seen w/ infiltrate. Kidney labs are abnormal w/ hematuria and proteinuria. He's also having trouble hearing. He has a SADLE NOSE. What dx is this? Is this pt ANCA pos or neg? What's happening to the small vessels?

Granulomatosis w/ Polyangitis (GPA) ANCA pos - C-ANCA/PR3-ANCA Necrotizing vasculitis

What ANCA is associated w/ Eosinophilic Granulomatosis w/ Polyangitis (EGPA)? Associated sxs?What's another name for this dx?

MPO-ANCA Granulomas w/ eosinphillia - asthma + eosinophillia -> vasculitis Churg-Strauss Syndrome

A young male < 35 yo presents c/o cool hands and feet. He smokes often. Diagnosis? What's the diagnostic feature and tx?

Thromboangiitis Obliterans (Buerger Dx) Angiography shows corkscrew appearance of vessels STOP smoking (only tx)

What's the difference between primary and secondary Raynaud Phenomenon? What's the progression or what the fingers look like?

Primary = benign, symmetric, response to cold or emotion Secondary = more severe ischemia, unilateral, drug-induced or other Pallor (vasoconstriction) > cyanosis (ischemia) > erythema (reperfusion)

If Giant Cell Arteritis (GCA) isn't treated, what can occur? What's tx?

Blindness Corticosteroids immediately!

What vessels does GCA involve? What are the sxs? What's a lab value commonly seen?

Large - cranial (temporal/facial/ophthalmic) - aortic arch Headache, jaw claudication (cramping d/t vasculature blockage), visual abnormalities) ESR inc (> 50 mm/h)

What is GCA associated w/ usually? Describe it.

Polymyalgia Rheumatica (PMR) - proximal symmetrical stiffness/soreness/pain in shoulder, neck and pelvic girdles - weakness is secondary to pain (pt can't do much d/t pain, mm fine) - no inflammation on m bx - Carpal tunnel syndrome associated!!!!!! - m enzymes and EMG normal - ESR and CRP elevated though!

What's the genetic component of GCA?

HLA-DR4

Who's most at risk for GCA?

W > 50 yo that have northern european ancestry

45 yo WF presents w/ CP. History of positive anti-Sm and Anti-DS DNA. On recent testing she had positive phospholipid antibodies. 2D echo reveals valve thickening and verrucous valvular lesions. What is this condition called?

Libman Sacks endocarditis

Connective Tissue Dx Flashcards

(47 cards)