Autoimmunity, Myopathies, SLE Flashcards
Name some organ specific types of autoimmune disease
The most at risk population for SLE is _
The most at risk population for SLE is women in reproductive years
Fill out this autoimmune ROS:
* Skin:
* Joints:
* Eyes:
* ENT:
* Serositis:
* Urinary:
* Neuro:
* Heme:
Fill out this autoimmune ROS:
* Skin: rashes
* Joints: arthralgia, joint swelling
* Eyes: dry eye, irritation
* ENT: dry mouth, oral ulcers
* Serositis: pleuritic chest pain
* Urinary: hematuria, frothy urine
* Neuro: headache, seizures, neuropathic sx
* Heme: clots, anemia
Signs of hemolytic anemia include:
1. _ Haptoglobin
2. _ Lactate dehydrogenase
3. _ RBCs
Signs of hemolytic anemia include:
1. Low Haptoglobin
2. Elevated Lactate dehydrogenase
3. Spherocytes RBCs
Autoantibodies _ and _ are most specific for SLE
Autoantibodies anti-dsDNA and anti-Smith are most specific for SLE
What are three major reasons of low complement?
- Chronic liver disease
- Use of complement (autoimmune)
- Deficiency
Autoimmune diseases/ Type III hypersensitivity can activate the _ complement pathway
Autoimmune diseases/ Type III hypersensitivity can activate the classical complement pathway
* Activated by antigen-antibody complex
Recall that the lectin pathway is activated by _
Recall that the lectin pathway is activated by microbial carbohydrates (ex: MBL)
Recall that the alternative pathway is activated by _
Recall that the alternative pathway is activated by activating surfaces
Therapy for acute flare of lupus is _ + _
Therapy for acute flare of lupus is corticosteroids + hydroxychloroquine
* We avoid long term use of corticosteroids
Name the various inflammatory myopathies
Dermatomyositis and polymyositis both occur in [populations] and have an acute to subacute presentation of weeks to months
Dermatomyositis and polymyositis both occur in children and adults and have an acute to subacute presentation of weeks to months
The muscle distribution affected in dermatomyositis and polymyositis is _
The muscle distribution affected in dermatomyositis and polymyositis is symmetrical, proximal involvement
* Classically affects shoulder and hip girdle and preserves distal muscles
* May cause dysphagia, weakness of respiratory muscles, heart involvement
Inclusion body myositis classically affects [population] and has _ onset
Inclusion body myositis classically affects older adults and has chronic, insidious onset (months to years)
The muscle distribution affected in inclusion body myositis is _
The muscle distribution affected in inclusion body myositis is asymmetric, proximal & distal muscle groups
* Does NOT spare distal muscle groups!
The confirmatory diagnosis for inflammatory myopathies is _
The confirmatory diagnosis for inflammatory myopathies is muscle biopsy
Inflammatory myopathies may present with elevated muscle enzymes, _ and _
Inflammatory myopathies may present with elevated muscle enzymes, CK and Aldolase
* Sometimes LFTs are elevated, esp AST
Common autoantibodies assocaited with dermatomyositis include:
Common autoantibodies assocaited with dermatomyositis include: anti-Jo-1, anti-Mi-2, anti-MDA5
Polymyositis involves infiltrative _ cells in the [muscle layer]
Polymyositis involves infiltrative CD8+ cells in the endomysium
* May see diffuse regions of necrotic and regenerating myofibers
Dermatomyositis involves infiltrative _ cells in the [muscle layer]
Dermatomyositis involves infiltrative CD4+ cells in the perimysium
* Inflammation and vasculitis leads to diminished blood flow to the peripheral muscle bundles –> perifascicular atrophy
Polymyositis
Dermatomyositis
Inclusion body myositis- CD8+ cells invading myofiber; presence of sarcoplasmic rimmed vacuoles; may see amyloid deposits
Patients with polymositis and dermatomyositis are at increased risk of [lung manifestation]
Patients with polymositis and dermatomyositis are at increased risk of interstitial lung disease
Name some extra-muscular manifestations in inflammatory myopathies
- Constitutional symptoms (fatigue, fevers, weight loss)
- Arthritis
- Gastroesophageal dysmotility
- Pulmonary hypertension
- Myocarditis
- Vasculitis (more prominent in pediatric population)
- Raynaud’s Phenomenon
- Increased risk for occult malignancy
First line pharmocologic treatment for inflammatory myositis is _ ; steroid sparing agents like _ can also be used
First line pharmocologic treatment for inflammatory myositis is corticosteroids ; steroid sparing agents like methotrexate can also be used
Name the cutaneous manifestations of dermatomyositis
Joint stiffness that is worse in the morning is more suggestive of _
Joint stiffness that is worse in the morning is more suggestive of inflammatory arthritis
* Inflammatory symptoms will get better with movement but mechanical ones get worse
Rheumatoid arthritis is a multi-system, [symmetry] inflammatory polyarthritis that predominantly affects the hands and feet early
Rheumatoid arthritis is a multi-system, symmetric inflammatory polyarthritis that predominantly affects the hands and feet early
The peak onset of RA is [age]
The peak onset of RA is 45-65
(RA/ osteoarthritis) is more likely to involve the DIP
Osteoarthritis is more likely to involve the DIP
* Both involve the PIP
(RA/ osteoarthritis) is more likely to involve the MCP
RA is more likely to involve the MCP
* Both involve the PIP
The classic autoantibodies involved in RA are _ and _ ; _ is more specific
The classic autoantibodies involved in RA are rheumatoid factor and anti-CCP ; anti-CCP is more specific
* A high anti-CCP titer is associated with more damage
Patients with [HLA] are more susceptible to RA
Patients with HLA-DRB1 are more susceptible to RA
RA nodules
[Eye manifestations] can occur in RA
Keratoconjunctivitis sicca, peripheral ulcerative keratitis, scleritis can occur in RA
[Renal disease] is a rare manifestation in RA patients
AA amyloidosis is a rare manifestation in RA patients
First line treatment for new onset active RA is _
First line treatment for new onset active RA is methotrexate
* Hydroxycloroquine, TNF inhibitors, IL-6 inhibitors, anti-CD20 Abs, JAK inhibitors also exist
Pulmonary manifestations of RA include _
Pulmonary manifestations of RA include ILD, pleuritis, pulmonary nodules
CV manifestations of RA include:
CV manifestations of RA include: pericarditis, coronary artery disease
Hematologic manifestations of RA include _
Hematologic manifestations of RA include anemia, thrombocytosis, leukemia/lymphoma
Sjogren’s disease is an autoimmune disease characterized by lymphocytic infiltration of _
Sjogren’s disease is an autoimmune disease characterized by lymphocytic infiltration of exocrine glands
* Associated with systemic disease and risk of lymphoma
_ is a test in which calibrated filter paper strip is placed in the lower eyelid of each eye, and the patient keeps their eyes closed for five minutes. The paper’s length that becomes wet is then measured
Schirmer’s test is a test in which calibrated filter paper strip is placed in the lower eyelid of each eye, and the patient keeps their eyes closed for five minutes. The paper’s length that becomes wet is then measured
The gold standard test for Sjogren’s is _
The gold standard test for Sjogren’s is salivary lip gland biopsy
* To identify lymphocytic sialadenitis
Sjogren’s disease is associated with a [type] RTA
Sjogren’s disease is associated with a distal Type 1 RTA
* Impaired distal tubule/ collecting duct acidification
* Alpha-intercalated cells are unable to secrete H+
* No new bicarb is generated leading to metabolic acidosis
* Bicarb will be low, K will be low
RA is caused by _ antibodies against the Fc portion of _
RA is caused by IgM antibodies (RF) against the Fc portion of IgG
Risk factors for RA include smoking, HLA _ or _
Risk factors for RA include smoking, HLA DR1 or DR4
Felty syndrome is a rare extra-articular manifestation of seropositive rheumatoid arthritis characterized by RA, _ and _
Felty syndrome is a rare extra-articular manifestation of seropositive rheumatoid arthritis characterized by RA, neutropenia and splenomegaly
_ is an extra-articular manifestation of rheumatoid arthritis characterized by pneumoconiosis
Caplan syndrome is an extra-articular manifestation of rheumatoid arthritis characterized by pneumoconiosis
_ is a joint deformity seen in RA in which there is PIP flexion and DIP hyperextension
Boutonniere deformity is a joint deformity seen in RA in which there is PIP flexion and DIP hyperextension
_ is a joint deformity seen in RA in which there is DIP flexion and PIP hyperextension
Swan neck deformity is a joint deformity seen in RA in which there is DIP flexion and PIP hyperextension
SLE is associated with HLA _ and _
SLE is associated with HLA DR2 and DR3
Msk effects of lupus include _
Msk effects of lupus include symmetrical polyarthritis with inflammation of the MCP + PIP without deformity
[Endocarditis] is a manifestation of lupus
Libman-Sacks endocarditis is a manifestation of lupus
Sjogren syndrome is associated with [gallbladder pathology]
Sjogren syndrome is associated with primary biliary cirrhosis
Sjogren syndrome is most commonly associated with [autoantibodies (2)]
Sjogren syndrome is most commonly associated with anti-Ro/SSA + anti-La/SSB
* Non-specific serology include rheumatoid factor, ANA, elevated CRP/ESR
Sjogren syndrome patients have a risk of [heme malignancy]
Sjogren syndrome patients have a risk of B cell non-hodgkin lymphoma like MALT lymphoma
[Inflammatory myopathy] involves cell-mediated cellular damage while _ involves antibody mediated cellular damage
Polymyositis involves cell-mediated cellular damage while dermatomyositis involves antibody mediated cellular damage
_ is associated with heliotrope edema
Dermatomyositis is associated with heliotrope edema
* Additionally, gottron papules, shawl and V signs
Patients with polymyositis or dermatomyositis should be tested for underlying _
Patients with polymyositis or dermatomyositis should be tested for underlying malignancy
Scleroderma is caused by an autoimmune non-inflammatory vasculopathy that leads to _ activation and _ deposition
Scleroderma is caused by an autoimmune non-inflammatory vasculopathy that leads to fibroblast activation and collagen deposition
Scleroderma is most common in [population]
Scleroderma is most common in women, 30-50 years old
Thickened skin, pruritus, loss of wrinkles, and telangiectasias should raise suspiscion for _
Thickened skin, pruritus, loss of wrinkles, and telangiectasias should raise suspiscion for scleroderma
Symptoms of limited scleroderma can be remembered with mneumonic _
Symptoms of limited scleroderma can be remembered with mneumonic CREST
* Calcinosis cutis
* Raynaud phenomenon
* Esophageal dysmotility
* Sclerodactyly
* Telangiectasia
_ form of scleroderma is a more benign course with less skin involvement and a slower onset
Limited scleroderma (CREST syndrome) form of scleroderma is a more benign course with less skin involvement and a slower onset
Diffuse scleroderma involves _
Diffuse scleroderma involves widespread skin thickening multiorgan involvement + life-threatening complications
Mixed connective tissue disease has overlapping characteristics of 3 syndromes:
Mixed connective tissue disease has overlapping characteristics of 3 syndromes: systemic sclerosis, SLE, polymyositis
[Autoantibody] is more specific to limited scleroderma
Anti-centromere is more specific to limited scleroderma
* Anti-Scl-70 occurs in both
[Autoantibody] is more specific to diffuse scleroderma
Anti-RNA polymerase III is more specific to diffuse scleroderma
* Anti-Scl-70 occurs in both
Mixed connective tissue disease is associated with [autoantibody]
Mixed connective tissue disease is associated with anti-U1 RNP antibody
Sarcoidosis involves the formation of _ secondary to antigen trigger and immune response
Sarcoidosis involves the formation of noncaseating granuloma formation secondary to antigen trigger and immune response
Formation of granulomas involves macrophages activating _ which then release _
Formation of granulomas involves macrophages activating Th1 which then release IFN-gamma
* Stimulates the formation of epitheloid cells
Hypervitaminosis D and hypercalcemia can occur in sarcoidosis due to increased activity of _
Hypervitaminosis D and hypercalcemia can occur in sarcoidosis due to increased activity of 1 alpha-hydroxylase
* Macrophage activity stimulates this enzyme –> increased calcitrol
Lupus pernio is a skin rash specific to _
Lupus pernio is a skin rash specific to sarcoidosis
Lofgren syndrome (sarcoidosis manifestation) involves fever + (3 symptoms)
Lofgren syndrome (sarcoidosis manifestation) involves fever +
1. Bilateral hilar lymphadenopathy
2. Erythema nodosum
3. Migratory polyarthritis
Sarcoidosis most commonly presents with _ on chest x-ray
Sarcoidosis most commonly presents with bilateral hilar lymphadenopathy on chest x-ray
* PFTs would show restrictive lung disease
Increased ACE levels in sarcoidosis result from increased formation of _
Increased ACE levels in sarcoidosis result from increased formation of epithelioid cells
Isolated sarcoidosis does not require treatment; however, symptoms may be treated with _
Isolated sarcoidosis does not require treatment; however, symptoms may be treated with glucocorticoids
RA patients are at an increased risk of [CV manifestations]
RA patients are at an increased risk of pericarditis and MI
A patient with a history of dry eyes, dry mouth, dental carries, oral candidiasis, and parotid swelling is concerning for _
A patient with a history of dry eyes, dry mouth, dental carries, oral candidiasis, and parotid swelling is concerning for Sjogren’s disease
A pregnant woman with a +SSA antibody is at risk of having a baby with _
A pregnant woman with a +SSA antibody is at risk of having a baby with neonatal lupus
* Manifestations: congenital heart block, elevated liver enzymes, cytopenias, rash
* However, a mother with Sjogren’s who is SSA+ and SSB+ still has a very low risk of neonatal lupus
Neonatal lupus is associated with _ rash, cytopenias, liver disease, and congenital heart block
Neonatal lupus is associated with “racoon eye” rash, cytopenias, liver disease, and congenital heart block
If a patient with Sjogren’s develops B symptoms or unilateral parotid gland swelling we should be concerned for _
If a patient with Sjogren’s develops B symptoms or unilateral parotid gland swelling we should be concerned for MALT lymphoma
* The most common lymphomas in these patients are marginal zone lymphomas like MALT
_ is a vasospastic reaction to cold temperatures or emotional stress that leads to sharply demarcated color changes in the skin
Raynaud phenomenon is a vasospastic reaction to cold temperatures or emotional stress that leads to sharply demarcated color changes in the skin
* Associations with multiple autoimmune conditions but most notably systemic slcerosis
The (3) phases of raynaud phenomenon are:
The (3) phases of raynaud phenomenon are:
1. Ischemia
2. Cyanosis
3. Rubor
Pharmacologic management of raynaud phenomenon includes:
Pharmacologic management of raynaud phenomenon includes:
* Calcium channel blockers
* Topical nitroglycerin (vasodilator)
* PDE inhibitors
_ Systemic sclerosis includes early, rapid visceral involvement, involvement of the trunk, ILD, renal crisis, and GI effects
Diffuse systemic sclerosis includes early, rapid visceral involvement, involvement of the trunk, ILD, renal crisis, and GI effects
Decreased renal blood flow from scleroderma results in overactivation of RAAS and a high _ state
Decreased renal blood flow from scleroderma results in overactivation of RAAS and a high renin state
* Scleroderma renal crisis = identical to malignant hypertension
Treatment for scleroderma renal crisis is _
Treatment for scleroderma renal crisis is ACE-inhibitors
* Scleroderma patients should check blood pressure at least once a week bc of risk
Limited cutaneous systemic sclerosis is associated with late development of [lung pathology]
Limited cutaneous systemic sclerosis is associated with late development of pulmonary arterial hypertension
* PAH much more common in limited
ILD is associated with the presense of [antibody] in scleroderma patients
ILD is associated with the presense of Scl-70 (topoisomerase 1) in scleroderma patients
* ILD is much more common in diffuse
_ syndrome is a pathognomonic presentation of sarcoidosis that involves anterior uveitis, parotid gland enlargement, facial palsy, and fever
Heerfordt’s syndrome is a pathognomonic presentation of sarcoidosis that involves anterior uveitis, parotid gland enlargement, facial palsy, and fever
Autoantibodies specific to SLE
Anti-dsDNA, anti-Smith
Autoantibody specific to drug-induced lupus
Anti-histone
Autoantibody specific to mixed connective tissue disease
Anti-U1 RNP
The most specific autoantibody to RA is _
The most specific autoantibody to RA is anti-cyclic citrullinated peptide (anti-CCP)
Rheumatoid factor is a _ antibody
Rheumatoid factor is a IgM antibody against IgG Fc region
Autoantibodies specific to Sjogrens
Anti-Ro (SSA) + Anti-La (SSB)
Diffuse scleroderma is associated with [autoantibody]
Diffuse scleroderma is associated with anti-Scl-70 + anti-RNA pol III
Anti-Scl-70 is a _ antibody
Anti-Scl-70 is an anti-DNA topoisomerase I
Limited scleroderma, CREST syndrome is associated with [autoantibody]
Limited scleroderma, CREST syndrome is associated with anti-centromere
Polymyositis and dermatomyositis are associated with [autoantibodies]
Polymyositis and dermatomyositis are associated with anti-synthetase (anti-Jo-1), anti-SRP, anti-helicase (anti-Mi-2)
Anti-SSA and anti-SSB are [type] antibodies
Anti-SSA and anti-SSB are anti-ribonucleoprotein antibodies
