Generalized Pain/Weakness Flashcards
What does CREST syndrome in limited scleroderma stand for?
Calcinosis, Raynaud’s Phenomenon, Esophageal Dysfunction, Sclerodactyly, Telangiectasis
What is the 1st line treatment for SLE?
Hydroxychloroquine
What is a Heliotrope rash pathognomonic for?
Dermatomyositis
What medication is added onto patients with dermatomyositis as opposed to polymyositis?
Hydroxychloroquine (Prednisone is indicated for both patients, but just initially for dermatomyositis. Then it is tapered and HCQ is used as mainstay)
What is the Gold Standard test to diagnose oral involvement of Sjogren syndrome?
Salivary gland biopsy
What differentiates polymyositis from dermatomyositis?
Polymyositis is mainly generalized muscle weakness alone
Dermatomyositis is generalized muscle weakness + classic skin findings (heliotrope rash, grotton’s papules, shawl sign, holster sign, v neck sign, flagellate erythema, poikiloderma, calcinosis cutis, nail fold changes)
What diseases are associated with Raynauds?
SLE, Scleroderma
What disease should you associate with dry mucosal membranes (dry eyes, dry mouth etc…)
Sjogrens
Name the special tests for ocular involvement that can help in the diagnosis of Sjogren Syndrome.
Schirmer test, Rose bengal test, slit lamp exam
What conditions are associated with an increased malignancy risk?
Sjogren Syndrome, polymyositis, dermatomyositis, maybe SLE
What syndrome is associated with the HLA-DR7 gene?
APS- Antiphospholipid Syndrome
What is required for diagnosis of Antiphospholipid Syndrome (APS)?
1 diagnostic lab criteria and 1 clinical manifestation
What are the most common side effects of hydroxychloroquine?
Ocular effects (many problems with the eyes)
What is the common clinical presentation of SLE that includes erythema over the cheeks and nose that spares the nasolabial folds?
Malar rash
What disease is associated with the feeling of a chronic ache that feels like you are “tied up in knots” and is accompanied by allodynia and hyperalgesia?
Fibromyalgia
What disorder is associated with a red-> white -> blue discoloration of the skin?
Raynaud Phenomenon
What immunologic findings are consistent with Sjogren’s syndrome?
anti-Ro/SS-A, anti-Ro/SS-B, RF, hypocomplementemia, cryglobulins, ANA
Which class of drugs is contraindicated for patients with Sjogren’s syndrome?
Anticholinergics (will dry them out even more!)
If a patient with antiphospholipid syndrome is pregnant and has a history of a thrombotic event, what would be the best treatment?
low molecular weight heparin + ASA throughout pregnancy until 6 weeks postpartum
What is the first line management for polymyalgia rheumatica?
Steroid taper (Predinsolone)
What is a complication of sjogren syndrome?
Increased risk for developing lymphoma
Anti-cardiolipin Ab,
Anti-Beta2 Glycoprotein Ab,
and Lupus anticoagulant in blood are diagnostic criteria associated with which diagnosis?
Antiphospholipid Syndrome (APS)
Of the (4) auto-antibodies associated with Scleroderma: ANA, Anti-Scl-70, Anti-centromere, Anti-RNA polymerase:
Which is associated with the Diffuse form?
Which is associated with the Limited/CREST form?
Which is associated with renal involvement?
Anti-Scl-70 (Diffuse)
Anti-centromere (Limited/CREST)
Anti-RNA polymerase (renal)
ANA (non-specific! but nearly all with scleroderma have)
What is the treatment for Severe, refractory SLE?
DMARDs + steroids
What can be prescribed for the symptomatic treatment for patients with ocular involvement in Sjogren Syndrome?
cyclosporine eye drops
Which diseases are associated with Raynaud’s phenomenon?
Scleroderma, Sjogren Syndrome, SLE, polymyositis
In which disease are you most likely to find elevated CK, ALT, AST and autoantibodies?
POLYMYOSITIS
Where are common locations for pain in patients with Polymyalgia Rheumatica?
Shoulders and pelvic girdle
What condition is CREST syndrome most commonly associated with?
Scleroderma
Secondary Raynaud’s syndrome is most often seen with with which other autoimmune disorder?
Systemic Lupus Erythematous
What diagnostic test is the gold standard for diagnosing polymyositis?
Muscle biopsy showing myocyte destruction and inflammation
What are some environmental factors that may contribute towards the development of SLE?
UV light, silica dust
What is the definitive way to diagnose dermatomyositis?
skin biopsy
What are the STOP considerations in regards to polymyalgia rheumatica?
S - SICK: increase steroid dose when sick
T - Treatment card: provide a card to alert others they are steroid dependent
O - Osteoporosis prevention w/bisphosphonate prophylaxis
P - Proton pump inhibitor
What is the current diagnostic criteria for fibromyalgia?
Widespread pain 3+ months not explained by another disorder
Widespread pain index > 7
Symptom Severity Scale > 5
When does scleroderma usually peak?
18-24 Months
What labs are typically elevated with polymyositis?
CK, AST, ALT.
Special Labs associated with Sjogren?
Anti-Ro and Anti-La
Common cause of miscarriages?
anti-phospholipid syndrome
Increased risk of what with patients with anti-phospholipid syndrome?
DVT/PE
What type of physical exam can help diagnose scleroderma, as it almost 100% present in a patient with the disease?
Nailfold capillary exam – will find dilated capillary loops.
What is the number one complaint for a patient with polymyositis?
weakness
What is the first line treatment for polymyositis?
Prednisone
if you have to treat Raynaud’s with medication what is going to be first line?
Calcium Channel Blockers
Your patient with scleroderma goes into renal crisis. What do you use to treat this?
ACE-I
What are the management goals for SLE?
Increase survival Decrease flares Prevent organ damage Limit drug toxicity Improve QOL
What is the prognosis for scleroderma?
Limited - 90% 5 year survival rate
Diffuse - 70-80% 5 year survival rate
What is a common complication of SLE that develops within the first 3 years and causes significant morbidity and mortalilty?
Lupus Nephritis
Which test will confirm the dx, evaluate severity, and guide tx for a patient with SLE that you suspect has lupus nephritis?
Renal biopsy
What are non-pharmalogic tx recommendations for Fibromyalgia?
pt education, graded exercise, heated pool, CBT, diet, and acupuncture
What are triggers for Raynaud’s?
Cold!! stress, nicotine, caffeine, decongestants
Which fibromyalgia grouping would a patient with high anxiety, low pain control and high tenderness with catastrophizing personality?
Group 2
AntiPhospholipid Antibody Syndrome (APS) can occur independently or in association with what other disease?
SLE
A abnormal clotting time that is corrected by the addition of excess phospholipids would indicate a positive _____?
Lupus Anticoagulant (seen in SLE and APS)
does limited scleroderma patients usually have a new onset or long history of raynauds?
limited –> long history of raynauds
diffuse –> new onset
what is the #1 differential diagnosis you need to rule out prior to diagnosing sjogrens syndrome?
diabetes —- bc patient complaining of increased thirst despite drinking water
What are the significant symptoms caused by Polymyalgia Rheumatica?
Stiff and painful neck, shoulders, and pelvic griddle
What does CREST syndrome stand for?
C- Calcinosis – calcium deposits in the skin
R- Raynaud’s phenomenon – Spasm of blood vessels in response to cold or stress
E- Esophageal dysfunction – Acid reflux and decreased motility
S- Sclerodactyly – thickening and tightening of the skin of the fingers and hands
T- Telangiectasias – dilation of capillaries causing spider looking red marks
How many of the diagnostic criteria need to be met for an SLE diagnosis?
4/11
Treatment for CNS manifestations of SLE?
high dose steroids + immunosuppressants
What is the differentiating characteristic of Morning stiffness in RA vs Fibromyalgia?
FM - morning stiffness NOT relieved by exercise
Which characteristic of Polymyalgia Rheumatica differentiates it within its umbrella condition, Giant Cell Arteritis?
No “above the neck” symptoms
What type of rash is most commonly associated with SLE?
Malar rash- erythema over the cheeks and nose
***spares nasolabial folds
What tests are specific and sensitive in detecting SLE?
dsDNA, anti-Sm
Venous thrombosis, arterial thrombosis, and pregnancy related complications are clinical manifestations of what disease?
Antiphospholipid Syndrome
Treatment for antiphopholipid syndrome in a patient who is pregnant with no history of thrombotic events (or only 1 preterm birth)
Avoid triggers that increase risk for clotting (smoking, estrogen birth control, etc)
ASA only after positive pregnancy test
What disorder is associated with xerophthalmia and xerostomia?
Sjogren Syndrome
What is the most common constitutional symptom of SLE?
Chronic fatigue
Possible complications of Raynaud’s
ulcers and amputation
True or false: A patient with APS should be referred to both rheumatology and hematology.
True.
What are two common physical exam findings in Dermatomyositis?
Heliotrope rash and Gottron’s papules
What is the difference between polymyositis and dermatomyositis?
Dermatomyositis is polymyositis WITH skin findings.
which two diagnoses exhibit photosensitivity?
SLE, dermatomyositis
what hormones (3) are associated with SLE?
estrogen, progesterone, prolactin
what are the 2 types of clinical features associated with APS
thromboses or pregnancy complications
4 P’s associated with acute limb ischemia in antiphospholipid syndrome -
pulseless, pallor, pain, paresthesia
what is the pathogenesis for scleroderma?
some linked HLA groups/genetic factors, combined with some type of trigger, such as environmental factors like silica dust exposure from mining/stone masons. causes excess collagen deposition, which leads to fibrosis and scarring.
what are Grotton’s papules? what could they suggest the patient have?
Grotton’s papules are atrophic dermal papules, which are flat topped and sometimes macules prior to raising. usually found over the MCP joint. over half of patients with myositis can have this.
What is the treatment for Antiphospholipid Syndrome?
Patients should be given Warfarin to maintain an INR of 2.0-3.0
What is the alternative treatment for APS if your patient is pregnant?
SubQ Heparin and 81mg Aspirin
What organ systems are affected in Mild SLE?
MSK and Skin
What can you expect to find on the labs of a pt with fibromyalgia?
Normal labs
Which population is least likely to have SLE
Blacks in Africa
Which populations are most likely to have worse renal prognosis in SLE
African Americans and Mexican Americans
Clinical Diagnosis of Sjogren’s Syndrome (4 out of 6 need to be positive)
Ocular symptoms – dry eyes, “feels like gravel in my eyes”
Oral symptoms – dry mouth; “drink a lot more often”
Ocular Signs – Schirmer or Rose Bengal Test findings
Histopathology – salivary gland biopsy
Salivary Gland Pathology
Antibodies to Ro/SS-A or La/SS-B antigens, or both
Most common epidemiology of a patient with SLE?
90% of SLE patients are females in their child-bearing years.
What is one way to differentiate between polymyositis and dermatomyositis during the physical exam?
Dermatomyositis will show cutaneous sign that precede or coincide with myositis (Gottron’s papules)
Sicca and xerostomia are classic clinical presentation in what rheumatologic disorder?
Sjogren’s Syndrome
Should a patient with lupus anticoagulant who has had no thrombotic event be put on anticoagulation?
No anticoagulation needed if has had no thrombotic event
What medications can be used with Raynauds syndrome/ disease if there is tissue destruction or severe disease?
Topical nitro, CCB (oral nifedipine), or sildenafil - all promote vasodilation to increase blood flow
Treatment for Lupus Nephritis in SLE
Cyclophosphamide
Difference between primary and secondary antiphospholipid syndrome?
Primary - no underlying cause
Secondary - other autoimmune disorders
Difference in SLE treatment for mild, moderate, severe?
Mild = HCQ + possible NSAIDs/Steroids Moderate = HCQ + steroids Severe = DMARDS + steroids
What INR range should a patient with active thrombosis in antiphospholipid syndrome be maintained within?
INR range of 2.0-3.0
Sjogren Syndrome need how many criteria for diagnosis
4/6 Ocular symptoms Oral symptoms ocular signs histopathology salivary gland involvement Abx to r/o other causes
Individuals most common affected by Dermatomyositis?
10-15 yo, 45-60yo, and females more than men
What groups are most affected by polymyalgia rheumatica?
Older people (>50), Caucasian, women>men
What are the first line treatments of Dermatomyositis?
Photoprotection (sunblock), Antimalarials (Hydroxychloroquine), systemic corticosteroids (prednisone)
