Al-Mehdi Pathogenetic treatment of CT disorders Flashcards by Madeline Wing

for most multisystem disorders, something in nucleus against which ______ are generated and cause disease

anti nuclear antibody (ANA)

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neutrophil has something in cytoplasm to which Ab’s are developed against

antineutrophil cytoplasmic antibody (ANCA)

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How to detect Ab’s

indirect immunofluorescence
ELISA (better specificity— higher predictive values)
Ab’s in dilution (titers)

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general fluorescence imaging of ANA’s is the first step in the diagnosis of what

CT disorders

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Female predominance (middle aged); autoimmune- autoantibodies against nucleic acids (nuclear antigens); deals w/ cell death of neutrophils by NET-osis

SLE (Lupus)

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specific autoantigens of SLE

ssRNA, dsRNA, DNA

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products of pro-inflammatory cytokines and type I interferons produce

inflammation

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2 main things that occur due to the immune response of body with SLE patient

vasculitis and inflammation

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vasculitis and inflammation can eventually lead to what that can lead to organs not functioning properly

tissue damage

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malar rash that does not cross nasolabial fold characteristic of what

SLE

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nephritis and pericarditis are also major features of what

SLE

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specific antinuclear antibodies for SLE

anti-dsDNA or anti-Smith

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in a female patient w/ lupus, what can cause spontaneous loss of pregnancy

antiphospholipids

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diagnosis

SLE (lupus)

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MoA for Tacrolimus and voclosporin to treat SLE

block IL-2

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MoA of hydroxychloroquine (HCQ) for treatment of SLE

targets TLR9 (blocks DAMP recognition on macrophages)

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2 main drugs that block PGE2 activity; and which one is better to treat SLE

celecoxib and aspirin(better)

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MoA of prednisone and methylprednisolone to treat SLE

inhibit gene expression of all cytokines

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MoA of belimumab and obinutuzumab and rituximab (the first two are best for SLE treatment)

block B cell activation

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The effector mechanisms that lead to tissue damage in autoimmune diseases are similar to those elicited in response to environmental antigens that result in allergy. Which hypersensitivity reaction is correctly matched to an autoimmune condition that occurs through a similar mechanism?

SLE and serum sickness (type III)

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multisystem noncaseating granulomatous disease

sarcoidosis

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HLA DQB1 and DRB1

sarcoidosis

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> 90% of patients w/ this disease exhibit mediastinal and hilar lymph node enlargement or lung disease

sarcoidosis

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autoantigens for sarcoidosis

ssRNA, dsRNA, DNA

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main organs involved in this disease include lungs, skin, and eyes

sarcoidosis

2 main outcomes of immune response in sarcoidosis

pulmonary fibrosis and vasculitis

50% of patients diagnosed with sarcoidosis by incidental what

CXR (chest xray)

lung involvement in >90% of patients with this disease; nonproductive cough

sarcoidosis

w/ sarcoidosis, what body part mainly experiences arthritis

ankles

Infiltrative skin lesions affecting the nose, cheeks, and ears in ___

chronic sarcoidosis

Lofgren syndrome in acute sarcoidosis

there is also increased_____ absorption in sarcoidosis

calcium

non-caseating granuloma in sarcoidosis

first line drugs for sarcoidosis

prednisone and ACTH

second line drugs for sarcoidosis

DMARDs

third line drugs for sarcoidosis

TNFa blockers (infliximab and adalimumab)

patient presents with painful lower extremity erythematous nodules and swelling and tenderness of ankles, what test should be ordered to support diagnosis

chest xray (for sarcoidosis

features of this include fibrosis and vasculopathy (thickening and fibrosis of skin)

systemic sclerosis

more prevalent in middle aged females and presents with thickened skin

systemic scleroderma

cause of death for patients w/ systemic scleroderma

pulmonary fibrosis and heart failure

CREST (anti-centromere Ab's) is diagnostic of what

systemic scleroderma

capillary loss and vasculopathy along w/ fibrosis is distinguishing for what

systemic scleroderma

the most characteristic and earliest vascular complication of systemic scleroderma

Raynaud's phenomenon

what do you not use to treat systemic scleroderma that could precipitate scleroderma renal crisis

glucocorticoids

main treatment for systemic scleroderma

immunotherapy

2 main types of drugs used to treat systemic scleroderma

antifibrotic and vascular drugs

D-penicillamine, pirfenidone, and nintedanib are what kind of drugs used to treat systemic scleroderma

antifibrotic

amlodipine and sildenafil are what type of drugs used to treat systemic scleroderma

vascular

bosentan and sildenafil are what type of drugs used to treat systemic scleroderma

vascular + PAH

Which of the following drugs may contribute to scleroderma renal crisis?

glucocorticoids

systemic sclerosis + SLE + Raynaud's

mixed connective tissue disease

autoantibodies against U1-RNP

mixed CT disease

one of the most common childhood disorders dealing with inflammatory arthritis in multiple joints

juvenile idiopathic arthritis

female predominance and ANA + for this

oligoarticular juvenile idiopathic arthritis

this has equal sex distribution and NO autoantibodies

systemic juvenile idiopathic arthritis

Most common rheumatic disease in pediatrics

Juvenile idiopathic arthritis (Still disease)

child presents with arthritis in multiple joints, fever w/ rash and hives and uveitis

juvenile idiopathic arthritis

treatment for this is targeted against IL-1 and IL-6

juvenile idiopathic arthritis

chronic autoimmune disorder; lymphocytic and plasma cell infiltration; destruction of salivary, lacrimal, and parotid glands

primary sjogren syndrome

most common of the autoimmune disease

primary sjogren syndrome

problem that presents with primary sjogren syndrome

risk of cancers

HLA-DRB1 antibodies against Ro/SS-A and la/SS-B

primary sjogren syndrome

due to T and B cell infiltration of exocrine glands

Sjogren syndrome

xerostomia and keratoconjunctivitus sicca

dry mouth and eyes for Sjogren syndrome

autoantibodies to SS-A and SS-B

Sjogren syndrome

to diagnose Sjogren

auto-Ab's against SS-A and SS-B and Schirmer test (tear test)

diagnosis

Sjogren syndrome

histopathology of primary Sjogren syndrome

treatment for dry eyes for Sjogren syndrome

Cyclosporine eye drops

treatment for dry mouth for Sjogren syndrome

Cevimeline

main diagnostic test to establish Sjogren syndrome diagnosis

Anti-Ro/SS-A antibodies

Which of the following is the best option for improving a patient’s ocular dryness in Sjogren syndrome?

cyclosporine eye drops

A 55-year-old woman is referred to your department for polysynovitis and palpable purpura with a Dx of Sjögren syndrome. Which of the following treatments would you recommend to treat these systemic manifestations?

Rituximab (b/c Sjogren deals with T and B cell infiltration)

this type of vasculitis is immune complex mediated

small vessel vasculitis

this type of vasculitis deals with immune complexes and anti-endothelial cell Ab's

medium vessel vasculitis

this type of vasculitis deals with granulomatous disease

large vessel vasculitis

giant cell arteritis and polymyalgia rheumatica are what type of vasculitis

large vessel

elevated ESR (inflammation) and temporal artery affected that can lead to blindess

large vessel vasculitis symptoms (specifically polymyalgia rheumatica)

to treat giant cell arteritis and polymyalgia rheumatica (large vessel vasculitis)

prednisone

pulseless disease and has multinucleated giant cell (large vessel vasculitis)

Takayasu arteritis

example of medium vessel vasculitis

Kawasaki disease

presents in children < 5 yrs, bilateral conjunctival congestion, buccal erythema, and strawberry tongue (can resemble toxic shock syndrome and scarlet fever)

Kawasaki disease

to treat Kawasaki disease

IVIG and ASPIRIN!!

medium vessel vasculitis

Polyarteritis Nodosa

Non-atherosclerotic, small and medium arteries, veins and nerves, <50 years old tobacco user, raynaud's (can lose your toes)

Thromboangitis obliterans (Buerger disease)

best treatment for Thromboangitis obliterans (Buerger Disease)

NO TOBACCO

Immunosuppressive therapy is known to be futile for which disease associated with vascular inflammation?

Buerger disease (thromboangitis obliterans)

ANCA associated vasculitis, strawberry gums, palpable purpura (small vessel vasculitis)

Granulomatous w/ polyangitis (Wegener Granulomatosis) GPA

ANCA associated vasculitis, tissue inflammation from eosinophils, targets peripheral nerves (small vessel vasculitis)

Eosinophilic Granulomatosis w/ polyangitis (Churg-Strauss syndrome) EGPA

to diagnose ANCA vasculitis GPA

c-ANCA (cytoplasmic)

to diagnose ANCA vasculitis (EGPA)

p-ANCA (perinuclear)

children, IgA vasculitis, palpable purpura

Henoch-Schonlein purpura

triad of oral and genital ulcerations and ocular involvement-uveitis (variable vessel vasculitis)

Behcet's syndrome

apthous ulcerations in the mouth (extremely painful); (variable vessel vasculitis)

Behcet's syndrome

to treat Behcet's syndrome

glucocorticoids and adalimumab

patient with Behcet's syndrome and already on anticoagulation meds and glucocorticoids should add what to his treatment

tumor necrosis factor inhibition (TNF)---adalimumab

blanching, cyanosis, and rubor of fingers or toes; vascular reactivity disorder

Raynaud disease

to treat Raynaud disease

sympathectomy (b/t T1 and T2)