Rheumatological Conditions Flashcards
(87 cards)
1
Q
what is the first line of autoantibody defense.
A
B cells develop in the Bone Marrow and any antibody created which would attack a body protein found in the bone marrow is destroyed.
2
Q
Without ____ of the B Cells, the body will not proliferate the autoantibody; this is the second line of defense against autoantibody production.
A
T Cell activation
3
Q
causes of autoimmunity
A
- genetics
- stress
- infection
- medication SE
4
Q
Rheumatology, probably more so than other fields of medicine, relies on recognition of _____ gleaned from a good H&P, labs, and imaging as many presentations will generate a large differential and labs and imaging are often non-specific
A
PATTERNS
5
Q
difference between highly sensitive test vs highly specific test?
A
- Highly Sensitive Test- Very few false neg; can cause false positives making healthy ppl being improperly dx; ability to R/O dx
- Highly Specific Test- Very few positive cases will be something other than what the tester is looking for; there are positives which are not picked up - false negatives may occur. People with disease may be missed; ability to rule IN dx
6
Q
the main lab to order if Suspect an autoimmune disease / Connective Tissue Disease?
A
ANA: Antinuclear Antibody
6
Q
what is an ANA: Antinuclear Antibody?
A
- Autoantibodies - antibodies that attack self
- Will attack the nucleus of healthy cells
7
Q
ANA is positive if titer is what ratio?
A
≥1:160
- Amount of saline dilution for antibodies to become undetectable
- ~5% of healthy ppl
- Higher titer = Greater likelihood; ≠ severity
- High Sensitivity; Low Specificity (more test needed)
- Once ANA is (+) - do not retest
8
Q
possible causes that increase ANA?
3
A
- infection
- CA
- pregnancy
9
Q
disease with (+)ANA based on sensitivity
A
- Drug-Induced Lupus (Hydralazine, Procainamide, Isoniazid) - 100%
- SLE - >95%
- Scleroderma - 85%
- Sjӧrgen Syndrome - 48%
- Dermatomyositis / Polymyositis - 61%
- RA - 40%
10
Q
patterns seen with SLE
5
A
- Anti-dsDNA - Rim
- Anti-SSA (Anti-Ro) - Speckled
- Anti-SSB (Anti-La) - Speckled
- Anti-Smith - SnRNP
- Anti-U1-RNP - U1-SnRNP
11
Q
pattern for Drug Induced Lupus
A
Anti-Histone, Homogenous
12
Q
Pattern for Progressive Systemic Sclerosis
A
- Anti-RNA-Polymerase - Nucleolar
- Anti-Scl-70 - DNA Top I
13
Q
patterns for Dermatomyositis
A
- Anti-Mi-2, Nucleolar
- Anti-Jo-1, Nucleolar
- Anti-SRP, Cytoplasmic
14
Q
patterns for Polymyositis
A
- Anti-Jo-1, Nucleolar
- Anti-SRP, Cytoplasmic
15
Q
pattern for crest syndrome
A
Anti-Centromere, Cytoplasmic
16
Q
pattern for Sjӧgren
A
- Anti-SSA (Anti-Ro), Speckled
- Anti-SSB (Anti-La), Speckled
17
Q
- Often a reflex lab with (+) ANA
- Leads the clinician to a more specific disease in the presence of possible Connective Tissue Disease
A
ENA: Extractable Nuclear Antigens
18
Q
Mixed Connective Tissue Disease is a term describing ?
A
overlap of clinical features of SLE, Systemic Scleroderma, and polymyositis.
19
Q
First detected in Rheumatoid Arthritis patients
Proteins in blood produced by immune system
RF presence indicates inflammatory process
which lab?
A
RF: Rheumatoid Factor
20
Q
RF: Rheumatoid Factor is found in what conditions?
A
- Rheumatoid Arthritis
- Sjogren’s Syndrome
- Juvenile Arthritis
- Scleroderma
- Mononucleosis
- TB
- Leukemia
- Multiple Myeloma
21
Q
2 most sensitive conditions to rheumatoid factor?
A
- Sjogren’s (75-95%)
- RA (70-90%)
not specific to RA
22
Q
what other infectious conditions may present with high rheumatoid factor?
A
syphilis, HCV, TB
23
Q
- A newer test more specific for Rheumatoid Arthritis than RF
- Is found in other diseases than RA, but at significantly lower levels.
A
Anti-CCP: Anti cyclic citrullinated peptide
- Sensitivity for RA - 66.0%
- Specificity for RA - 90.4%
24
Best at identifying Acute Inflammation
Produced by liver (not reliable test w/ liver failure)
CRP: C-reactive Protein
25
* Best at identifying Chronic Inflammation - Infection, Malignancy, Vasculitis, most Anemias, MI, Inflammatory Arthritis
* Normal test r/o temporal arteritis and polymyalgia rheumatica
* ↑ w/ age, pregnancy (2nd/3rd TM), medications (OCP)
ESR: Erythrocyte Sedimentation Rate
26
Two types of test of ANCA
* FA, immunofluorescence, (more sensitive)
* ELISA (more specific)
27
p-ANCA (Perinuclear pattern) is MC in what conditions
SLE, Sjogren's, RA, PM, DM
28
c-ANCA (Cytoplasmic pattern) is MC in what conditions
(+) Wegener Granulomatosis & Churg-Strauss Syndrome
29
A chronic, systemic, multi organ autoimmune disease of connective tissues secondary to antibody formation and immune complex deposition.
Systemic Lupus Erythematosus (SLE)
30
SLE MC affects who?
* female
* 16-55 y/o
* Native American > African American > Asian (Chinese, Filipino) > Hispanic and Japanese > Caucasian
31
Precipitating factors of SLE include:
* Sun exposure
* Stress
* Infections
* Pregnancy
* Surgery
32
Low grade fever
Joint Pain
Rash - Covers the cheeks & nasal bridge; spares the nasolabial folds
SLE
*Malar (butterfly) rash*
33
criteria to dx SLE
4 out of 11 common s/s:
1. Malar Rash
1. Discoid Rash
1. Photosensitivity
1. Oral Ulcers
1. Arthritis
1. Kidney dz
1. Neuro dz (In absence of lyte abnormalities or med SE)
1. Serositis - pericarditis, pleuritis
1. Hematologic dz - hemolytic anemia w/ reticulocytosis, lymphopenia, thrombocytopenia
1. Immunologic Abnormalities
34
immunologic dx criteria for SLE
1. abnml **ANA**
1. **Anti-dsDNA** in abnml titer OR
1. **Anti-Smith** OR
1. **(+) Antiphospholipid Ab**:
- abnml level IgG or IgM anticardiolipin ab OR
- (+) lupus anticoag using standard method OR
- **false (+) for syphilis** known to be positive for at least 6 mo and _confirmed by Treponema pallidum immobilization or fluorescent treponemal ab absorption test_
35
findings that indicate kidney disease
* >500mg protein in 24 hrs.
* ≥3+ protein UA dipstick
* Casts: Red, Hgb, granular, tubular, mixed
36
what ANA ratio is needed to use the ACR/EULAR 2019 criteria?
points needed?
≥ 1:80
10 points needed for inclusion.
37
derm manifestations of SLE
| 6
1. Malar Rash
1. Discoid Rash
1. Photosensitivity
1. Oral / Nasopharyngeal Ulcers
1. Raynaud’s
1. Alopecia
38
MSK manifestations of SLE
1. Arthritis - ≥2 joints, Migratory / Symmetric, Often peripheral, esp. hands
1. Myalgias
39
CV and pulm manifestations of SLE
1. Pericarditis or Pleuritis
1. Myocarditis
1. Valvular dz
1. Vasculitis
1. Interstitial Lung Disease
1. Pulmonary HTN
40
renal manifestations of SLE
**Lupus nephritis**
* **_BX_ proven Lupus Nephritis _guarantees SLE_**
* Hematuria
* Proteinuria
* Elv Creatinine
* May be asx - UA / BMP if considering SLE Dx
41
neuro manifestations of SLE
* Cognitive dysfunction
* Stroke
* Neuropathy
* Transverse myelitis
* HA
* Delirium
* Depression
42
GI manifestations of SLE
*Not on the list of 11*
* Dysphagia, abdominal pain, nausea
* Pancreatitis, gastritis, mesenteric vasculitis
43
systemic manifestations of SLE
Not on the list of 11
* Fatigue, fever, malaise, weight changes
* weakness
44
what is the most common manifestatio of SLE
MSK - 90%
45
what is drug-induced lupus?
* Strong Genetic predisposition
* resolves with DC of offending agent
* Not associated w/ CNS/Kidney/Alopecia
* **ANA+** - 100% sensitivity
* **(+) Anti-Histone Ab** - 95%
46
common medications that causes SLE
* (High Risk >5%) **Procainamide, hydralazine**, Penicillamine
* (Moderate Risk 1-5%) Quinidine
* Many other Low risk medications…
47
monitoring for SLE
* q 6 mo if completely stable; 3-4 mo
* Complete H/PE
* CBC: thrombocytopenia (acute flare); Anemia
* CMP
* UA w/ micro
* +/- ESR, CRP, Complement levels, Anti-dsDNA - fluctuate with flares
48
nonpharm mgmt for SLE
* refer
* psychological
* sun protection & **SPF >55**; **avoid meds w/ photosensitivity**
* diet: balanced, **vit D supp**, daily vitamins if GI concerns
* exercise
* smoking cessation
* immunization: flu, pneuomococcal, HPV, HBV
* **avoid pregnancy during flares** - **“dormant” x 6 mo before trying**
49
what medications to avoid in SLE
1. Sulfa Antibiotics (Bactrim) - rash in >30%,
1. Minocycline
50
mgmt for mild SLE
*Skin joint and mucosal*
* Hydroxychloroquine, (+/-) NSAID
* < 7.5 mg prednisone daily if needed
51
mgmt for moderate SLE
*Significant but non-organ threatening disease*
* Hydroxychloroquine + NSAID
* 5-15 mg prednisone daily, tapered
* Immunosuppressive (MTX or Azathioprine)
52
mgmt for severe SLE
*Renal/CNS involvement*
* Hydroxychloroquine + NSAID
* High dose IV prednisone
* Immunosuppressive agent (Belimumab, Rituximab, Cyclosporine)
53
possible tx options for joint sx in SLE
1. **Hydroxychloroquine** (Plaquenil)
2. Naproxen
3. Prednisone
54
which joint symptom meication is known to cause the “Bull’s Eye Retinopathy”
what is this called?
* Hydroxychloroquine
* Chloroquine Retinopathy
55
epidemiology of scleroderma
* 1-2/100,000 in USA
* Females
* MC 30-50 years of age
56
skin manifestations of scleroderma
1. puffiness
2. sclerodactyly
3. rest of body tightens
4. pigmentation changes and itching
57
vascular manifestations of scleroderma
1. _raynauds - 95%_
2. fingertip ulcerations
3. cutaneous telangiectasia
4. CAD
58
GI manifestations of scleroderma
1. GERD, hoarsenss, dysphagia
2. dyspepsia, early satiety, bloating
3. constipation, fecal incontinence
4. malnutrition and chronic iron def anemia
59
rsp manifestations of scleroderma
1. dyspnea - progressive
2. chest pain - pulm HTN
3. cough, dry - restrictive lung disease
60
MSK manifestations of scleroderma
1. arthralgia, myalgia
2. Loss of ROM, muscle weakness
3. carpal tunnel
61
cardiac manifestations of scleroderma
1. CHF
2. arrhythmias, palpitations
3. systemic sclerosis - independent risk for MI
62
renal manifestations of scleroderma
1. HTN
2. AKI
3. CKD
63
GU manifestation sof scleroderma
1. ED
2. dyspareunia
3. vaginal narrowing, dryness
64
ENT manifestations of scleroderma
1. sicca syndrome w/ poor dentition
2. tongue CA
3. blindess - retinal artery occlusion
| sicca: lymphocytic infiltration of salivary and lacrimal glands
65
neuro manifestations of scleroderma
1. TN
2. peripheral neuro
3. HA and CVA
66
There are at minimum 4 subsets of Scleroderma:
1. _Limited Cutaneous Systemic Sclerosis_ - s/s distal to the elbows and the face and neck. Many manifest CREST.
1. _Diffuse Cutaneous Systemic Sclerosis_ - proximal and even truncal manifestations.
1. _Systemic Sclerosis Sine Scleroderma_ - other s/s present w/o skin manifestations - _RARE_
1. _Systemic Sclerosis w/ Overlap Syndrome_ - Any of the above w/ overlap features of another systemic rheum dz (ex: SLE, Sjogren’s, RA, etc…)
67
what is CREST syndrome?
1. calcinosis
2. raynauds
3. esopahgeal dysfunction
4. sclerodactyly
5. telangiectasias
68
difference between CREST vs systemic
CREST - mainly head and hands
systemic - includes trunk and proximal extremities
69
MC GI manifestation of scleroderma
**esophageal** (90%)
Other GI sx:
* GERD
* Choking
* Cough after swallowing
* Bloating
* Early Satiety
* Dysphagia
70
w/u for scleroderma
* CBC - anemia, dec MCV (iron def and/or blood loss)
* BMP - dec kidney function
* **ANA (+)**
* **_Anti-DNA top I (Scl-70)_** - diffuse cutaneous SSc - risk of **lung dz**
* **_Anti-Centromere_** - assoc w/ **CREST**
* **_Anti-RNA polymerase III_** - Diffuse dz - risk of **renal failure** and **CA**
71
All patients should have baseline imaging/testing to determine if ____ ____ _____ is present
what are these testings?
| scleroderma
Interstitial Lung Disease
* PFTs
* Echo
* CT of Chest
72
tx for scleroderma
1. No Definitive Cure
1. Usually Organ System based
1. Raynaud’s - CCB, GERD - PPI
1. Arthritis: Similar to lupus
- NSAID (If kidneys stable)
- Inflammatory (RA), add in stepwise approach - Low dose steroids, Hydroxychloroquine, MTX, Biologics
1. PT and wt loss
2. Diffuse skin sclerosis or organ involvement - MTX: 15-25 mg weekly
3. REFER to Rheumatology!!!
73
chronic autoimmune inflammatory disorder characterized by diminished lacrimal and salivary gland function with resultant dryness of the eyes and mouth
Sjӧgren’s Syndrome
74
epidemiology of Sjӧgren’s Syndrome
* one of the more common autoimmune disorders
* Female
* 50-60 y/o
75
pathophys of Sjӧgren’s Syndrome
Ab attack:
* **exocrine glands** - xerostoma, dry eyes, parotid gland enlargement
* **endocrine glands** - thyroid dysfunction
* **extraglandular** - arthritis, GI, ILD, neuropathies
76
if secondary Sjӧgren’s Syndrome, it is often associated with what other conditions? (2)
1. RA
2. SLE
77
* Dry eyes - burning, itching, difficulty with contacts, ropy secretions, FB sensation, photophobia
* Dry Tongue - Halitosis, insatiable thirst, cavities - often at the gum line, dysphagia without liquid,
* Dry skin, vagina, nose, throat, larynx
dx?
w/u?
**_Sjӧgren’s Syndrome_**
* Basic labs - pancytopenia, hypokalemia, low bicarb, inc AST/ALT possible
* Elevated ESR (CRP is not)
* **ANA (-)**
* **Anti-SSA (Anti-Ro) / Anti-SSB (Anti-La) (+)** in primary disease
* **(+) HLA-DR52 gene**
* **Labial salivary gland bx** may confirm
* eye sx - **Schirmer Test & surface staining**
78
what other conditions may mimic sjogren's syndrome?
HIV, HCV, and Vit A def
79
tx for Sjӧgren’s Syndrome
1. **Eye drops**
1. Avoid smoking
1. **Omega-3 Fatty Acids**
1. Counsel women that SS is associated with congenital heart block if high titers of Ro/La
1. **Lotion** for skin
1. **Dental exams q 6 mo w/ fluoride tx**
1. **Pilocarpine**: Cholinergic
- Xerostomia: 5 mg PO QID
80
* SE of pilocarpine?
* what other med can help with SE?
diaphoresis, flushing, sweating, bradycardia, diarrhea, N/V, incontinence, blurred vision. (Cyclosporine may help with blurred vision)
81
Epidemiology of Polymyositis / Dermatomyositis
* DM MC > PM
* females
* DM in childhood or adulthood vs PM in adulthood
82
s/s of DM and PM
1. Insidious onset
1. **symmetric, progressive, painless *proximal* weakness**, eye and facial muscles spared, +/- dysphagia; extramuscular manifestations (ILD, polyarthritis, *skin changes in DM, _not PM_*)
1. Pain possible
1. Muscle Atrophy: difficulty rising from a chair, climbing steps, ADLs, etc
1. Overlap with other autoimmune disease possible
83
DM and PM have an Increased risk of ? 3-5 years after onset
* **cancer**
* **DM** > PM
*Ovarian, breast, colon, melanoma, Non-hodgkin’s*
84
Skin manifestations common with DM
1. **Gottron’s Papules** - raised, violaceous scaly eruptions on knuckles
1. **Heliotrope Eruption** (Blu-purple)
1. **Mechanic Hands** - hyperkeratotic with dirty appearance
1. **Shawl Sign**
85
w/u for Polymyositis / Dermatomyositis
1. Basic labs
1. **elevated ESR** (CRP is not)
1. **Muscle Enzymes** - Aldolase, CK (>10x ULN in active disease, inc CK-MB w/o Troponins)
1. **ANA (+)** MC
1. **Anti-Mi-2: DM**
1. **Anti-Jo-1: DM/PM** - Interstitial Lung Fibrosis, Mechanic Hands
1. **Anti-SRP: DM/PM** - _More exclusive to PM_, MSK weakness and cardiac s/s
1. Imaging
- MRI: Muscle edema or atrophy, fibrosis
- EMG: Myopathic Potentials
1. Muscle Bx - not necessary
86
tx for Polymyositis / Dermatomyositis
1. CA screening + CT chest, abd, pelvis, TVUS, Mammogram - **q 2 years**
1. _Swallow evaluation if dysphagia_
1. **Prednisone** 1 mg / kg [max = 100 mg/QD]
- Taper after 4-6 weeks
- **+/- DMARD (MTX / Sulfasalazine)**
- _topicals for skin_
- _PT for muscle weakness_
