Lupus and Rheumatic Disorders Flashcards
What is systemic lupus erythematosus (SLE)?
chronic multisystemic inflammatory autoimmune disease
Cause of SLE?
unknown
Process of SLE
abnormal immune response -> pathogenic autoantibodies -> deposit in tissue
What autoantibodies are produced in SLE?
antinuclear; antiphospholipid; anti-double-stranded DNA
x% of SLE patients have a family history
10%
Genes affected in SLE have impact on?
innate and/or adaptive immune responses; either increased activation or impaired regulation
Environmental factors of SLE
UV light; drugs (procainamide, hydralazine, quinidine, isoniazid)
Immunologic triggers of SLE
epstein barr, CMV, dietary factors (amino acid L-canavanine)
Hormonal triggers of SLE
strong link to females
Does SLE impact men or women more?
women
Key age of SLE
15-44
constitutional symptoms of SLE
fatigue, malaise, weight loss, fever, lymphadenopathy
skin/hair symptoms of SLE
malar rash, discoid rash, photosensitive rash, Raynaud’s phenomenon, alopecia
MSK symptoms of SLE
polyarthritis, polyarthralgias, myositis
kidney symptoms of SLE
HTN, proteinuria, hematuria, renal failure
CNS symptoms of SLE
seizures, psychosis, stroke, depression, HA
Cardiopulmonary symptoms of SLE
pericarditis, myocarditis, conduction abnormalities, pleuritis, parenchymal lung disease, pulmonary thrombosis, accelerated atherosclerosis with vascular endothelial dysfunction
GI symptoms of SLE
hepatosplenomegaly, pancreatitis, bowel vasculitis
Hematologic system symptoms of SLE
hemolytic anemia, thrombocytopenia
ocular symptoms of SLE
retinal vasculitis, scleritis
Is SLE erosive or non-erosive?
non-erosive
number of joints SLE impacts?
2 or more peripheral joints
Is SLE symmetrical or asymmetrical joint pain?
Symmetrical
Common SLE impacted joints
wrist, MCP, PIP
What’s a key MSK finding that may make you think SLE?
pain/stiffness out of proportion to PE
When do kidney manifestations commonly occur with SLE?
1st two years of onset
SLE can lead to what in the kidneys?
renal insufficiency and failure
How to determine if SLE has impacted the kidneys?
renal biopsy
What are the two major causes of SLE death?
kidney failure, accelerated atherosclerosis with vascular endothelial dysfunction
How many neuropyschiatric syndromes are associated with SLE?
19
What’s the most common neuropsychiatric symptom of SLE?
cognitive impairment
Which two neuropsychiatric symptoms are part of ACR Lupus diagnostic criteria?
seizure, psychosis
11 features of ACR SLE criteria
malar rash, discoid rash, photosensitivity, oral ulcers, arthritis, serositis, renal disorder, neurologic disorder, hematologic disorder, immunologic disorder, antinuclear antibody
HOw many ACR SLE features do you need to be diagnosed?
4/11
Describe renal disorder ACR SLE classifications
proteinuria (>3+) or cellular casts (red cell; hemoglobin; granular; tubular or mixed)
Describe neurologic disorder ACR SLE classifications
seizures or psychosis
Describe hematologic disorder ACR SLE classifications
hemolytic anemia with reticulocytosis; leukopenia; lymphopenia; thrombocytopenia
Describe immunologic disorder ACR SLE classifications
ANA to double-stranded DNA; positive antibody to Smith nuclear antigen; positive antiphospholipid antibody
Labs for SLE work up
CBC with diff, CMP, ESR, CRP, PT, PTT, ANA, urinalysis
What will you see in the CBC with diff of a SLE patient?
anemia, leukopenia, lymphopenia, thrombocytopenia
95% specificity for SLE; fluctuates with dz; assc with glomerulonephritis: test
Anti-dsDNA
99% specific for SLE: Test
Anti-Smith
Mixed connective tissue disease: test
Anti-U1RNP
Sjӧgren’s syndrome, photosensitivity: test
Anti-Ro/SSA
Sjӧgren’s syndrome: test
Anti-La/SSB
Drug induced lupus test
Antihistone
Arterial and venous thrombosis; pregnancy morbidity: test
Antiphospholipid
Two tests with highest prevalence in SLE
antihistone (70%); anti-dsDNA (60%)
Imaging for SLE workup
plain films of joints; CXR or Chest CT; ECG/Echocardiogram, renal biopsy
What are you looking for in CXR of SLE?
pleural effusion, pulmonary infiltrates, interstitial lung disease
What are you looking for in ECG/Echocardiogram of SLE?
pericarditis, pericardial effusion
What are you looking for with renal biopsy of SLE?
definitive diagnosis for lupus nephritis
What’s the leading cause of m/m in SLE?
atherosclerosis
What should SLE patients stop?
smoking
Conservative lifestyle modifcations for SLE?
avoid sunlight/high SPF sunscreen, avoid tobacco, avoid fatigue, adequate sleep
4 main SLE medication categories?
antimalarials, NSAIDs, corticosteroids, DMARDs
SLE 5 year survival rate?
90%
SLE 15 year survival rate?
85%
What’s Sjӧgren’s Syndrome?
Autoimmune disorder affecting exocrine glands; lymphocytic and plasma cell infiltration (salivary and lacrimal glands)
Sjӧgren’s Syndrome is associated with?
human leukocyte antigen (genetics)
Secondary Sjӧgren’s Syndrome is associated with?
other autoimmune connective tissue diseases
Etiology of Sjӧgren’s Syndrome
unknown
Environmental triggers of Sjӧgren’s Syndrome
viruses (maybe)–EBV, retroviruses, coxsackieviruses, CMV, Hep C; hormones
Who is impacted more by Sjӧgren’s Syndrome men or women?
women
When does Sjӧgren’s Syndrome peak?
4th and 5th decade
CM of Sjӧgren’s Syndrome
insidious onset, xerophthalmia (dry eyes), xerostomia (dry mouth), bilateral parotid gland swelling
Xerophthalmia in Sjӧgren’s Syndrome can lead to?
Keratoconjunctivitis sicca, corneal ulcers and scarring, bacterial keratitis, eyelid infections, visual impairment
Xerostomia in Sjӧgren’s Syndrome can lead to?
difficulty swallowing, altered sense of taste, dental caries/infections, fungal infections (candidiasis), pain with eating salty/spicy foods, difficulty talking/develop hoarseness, parotitis
How is sicca syndrome associated with Sjӧgren’s Syndrome?
it’s independent of Sjӧgren’s Syndrome
What is sicca syndrome?
dryness of exocrine glands from a variety of causes
Constitutional symptoms of Sjӧgren’s Syndrome
fatigue; low-grade fever
Skin symptoms of Sjӧgren’s Syndrome
xerosis, palpable purpura, Raynaud’s phenomenon, photosensitivity
pulmonary symptoms of Sjӧgren’s Syndrome
dry cough, chronic bronchitis, bronchiectasis, COPD
MSK symptoms of Sjӧgren’s Syndrome
Polyarthritis; polymyositis
Renal symptoms of Sjӧgren’s Syndrome
Tubular interstitial nephritis; glomerulonephritis; renal calculi
Neurologic symptoms of Sjӧgren’s Syndrome
Peripheral neuropathy; optic neuropathy; myelopathy
Neoplastic symptoms of Sjӧgren’s Syndrome
Lymphadenopathy; lymphoma; Non-Hodgkin’s lymphoma
Sjӧgren’s Syndrome work up
ocular and oral exams, Schirmer test, Rose Bengal staining, labs
Labs for Sjӧgren’s Syndrome
CBC with diff, CMP, RF, ANA, anti-ss-A (rho), anti-ss-B (La), elevated ESR; salivary gland biopsy maybe
What will the lab work look like for Sjӧgren’s Syndrome?
+RF; + ANA, + anti-ss-A (rho), +anti-SS-B (La), elevated ESR
6 diagnostic criteria for Sjӧgren’s Syndrome
ocular symptoms; oral symptoms; ocular signs; oral signs; positive minor salivary gland biopsy findings; positive anti-SSA or anti-SSB antibody results
What are the specific ocular symptom criteria for Sjӧgren’s Syndrome
dry eyes > 3 months; FBS; use of AT >3x a day
What are specific oral symptom criteria for Sjӧgren’s Syndrome?
dry mouth >3 months, recurrent swollen salivary glands, use of liquids to aid swallowing
What are specific ocular sign criteria for Sjӧgren’s Syndrome
positive test for schirmer or rose bengal stain
What are specific oral sign criteria for Sjӧgren’s Syndrome
abnormal salivary scintigraphy, parotid sialography, abnormal salivary flow
How many criteria do you need for Sjӧgren’s Syndrome diagnosis?
4+
Treatment for Sjӧgren’s Syndrome (non-rx)
fluid replacement, skin lotion, humidifiers, proper oral hygiene, annual eye/dental exams, treat secondary symptoms
Rx treatment for dry eyes (Sjӧgren’s Syndrome)
artificial tears, topical steroids, cyclosporine ophthalmic (restasis), lacrimal puncta plugging, glasses with moisture shields
Dry mouth treatment (Sjӧgren’s Syndrome)
encourage increased fluid intake, humidifiers, artificial saliva (salivart, saliment, mouthkote)
What is polymyalgia rheumatica?
Inflammatory condition affecting shoulders and pelvic girdle; non-erosive synovitis and tenosynovitis
Etiology of polymyalgia rheumatica?
unknown; associated with HLA-DR4 (activates immune system leading to inflammation)
Does polymyalgia rheumatica impact men or women more frequently?
women
CM of polymyalgia rheumatica
sudden onset of muscle pain and stiffness; neck, shoulders, arms; pelvic girdle and thigh muscles; possible muscle weakness; possible fatigue/malaise/loss of appetite
What condition is polymyalgia rheumatica associated with?
giant cell arteritis
giant cell arteritis targets?
medium to large extracranial blood vessels
Commonly affected arteries by giant cell arteritis
superficial temporal, ophthalmic, occipital, vertebral, posterior ciliary, proximal vertebral
RF for giant cell arteritis
women, age
S/S of giant cell arteritis
HA, TTP over temporal artery, visual changes, possible fatigue/malaise/fever
laboratory testing for polymyalgia rheumatica
ESR, CRP, CBC with diff, RF or anti-CCP, creatine phosphokinase, GCA-temporal artery biopsy
What’s the gold standard for GCA?
temporal artery biopsy
What do you expect with the ESR in polymyalgia rheumatica?
> 40
polymyalgia rheumatica diagnostic criteria
morning stiffness >45 minute (2 pts); hip pain/limited ROM (1 point); absence of RF and/or anti-CCP (2 points); absence of peripheral joint pain (1 point)
Treatment of polymyalgia rheumatica
prednisone 12.5-25 mg/day (long term add PPI to protect GI); Ca/Vit D
Treatment of giant cell arteritis
Prednisone 40-60 mg x 4 weeks; then decrease 10 mg every 2 weeks until reach 20mg, then reduce by 2.5 mg every 2-4 weeks to 10 mg; then decrease 1 mg every 1-2 months as long as no relapse
What is polyarteritis nodosa?
systemic vasculitic syndrome; Necrotizing inflammation of arterial media and inflammatory cell infiltration
What vessels does polyarteritis nodosa impact?
small and medium
Where in the vessel does polyarteritis nodosa impact?
bifurcations
What happens when vessels weaken in polyarteritis nodosa?
aneurysms, thrombi development, leads to hemorrhage and organ ischemia or infarction
Etiology of polyarteritis nodosa?
unknown, maybe Hep B/C, CECR1 gene mutation (encodes for adenosine deaminase 2 -> affects immune system)
Does polyarteritis nodosa impact men or women more frequently?
men
Polyarteritis nodosa is associated with?
hairy cell leukemia
Constitutional symptoms of polyarteritis nodosa?
fever, malaise, fatigue, anorexia, myalgia
NS symptoms of polyarteritis nodosa?
transient cerebral ischemia and monocular blindness, peripheral neuropathy
cutaneous symptoms of polyarteritis nodosa?
rash, purpura, gangrene, nodules, livedo reticularis, raynaud’s
What’s livedo reticularis?
common skin finding consisting of a mottled reticulated vascular pattern that appears as a lace-like purplish discoloration of the skin.
What condition is livedo reticularis associated with?
polyarteritis nodosa
GI symptoms of polyarteritis nodosa?
postprandial abdominal pain, N/V, organ infarction
renal symptoms of polyarteritis nodosa?
renal failure
labs for polyarteritis nodosa
CBC with diff, CRP, ESR, liver function, HBV/HCV serology, RF (negative), ANA (negative), angiography (aneurysmal dilation–renal, mesenteric, or hepatic arteries), biopsy, abdominal CT
Diagnostic criteria of polyarteritis nodosa (3/10)
Weight loss > 4 kg, Livedo reticularis, Testicular pain/tenderness, Myalgias, weakness or leg tenderness, Neuropathy, Diastolic bold pressure > 90 mmHg, Elevated BUN > 40 mg/dl or creatinine >1.5 mg/dl, Positive Hep B virus, Arteriography with aneurysms, Arterial biopsy with necrotizing inflammatory infiltrate
Treatment of polyarteritis nodosa?
prednisone 1-2 mg/kg/day (start higher and taper), maybe methotrexate with steroids, anti-viral for hep b, treat HTN
Prognosis of polyarteritis nodosa?
if untreated 5 year survival rate <20%; if treated increases to 50%
