RHeum Flashcards
Percentage of patients WITH the disease who have a Positive Test Result
Sensitivity
Percentage of patients Without disease with Negative Test Result
Specificity
What is the classic threshold for an elevated ESR
ESR > 100 generally means significant cancer, serious infection, renal or autoimmune disease
Is CRP relevant in Lupus
No
In chronic rheum d/o what will the complement be..
Hypo
Define spectrum of dz characterized by inflammation of the sacroiliac (SI) joints, spine, or peripheral joints
Spondyloarthropathies or Spondyloarthritis (SpA)
What is the hallmark feature of Ankylosing Spondylitis
Hallmark feature is LBP which improves with activity or exercise, worsens with rest
- Awakens pt at night
- Generally younger than 45 years of age
+Chest Pain
Eye S/s with ankylosing spondylitis
Acute anterior uveitis (inflammation of the iris, ciliary body & choroid) present in up to 50% over the disease course
Most common extra-articular manifestation
Typically abrupt, unilateral, intense pain, redness, photophobia
TTP over the sacral/illeal joint
Think
Ankylosing spondylitis
What would you expect to see on labs in a pt with Ankylosing Spondylitis
CRP elevated in 30-50% of patients with ankylosing spondylitis
Negative RF
Mild, normocytic anemia of chronic disease
Elevated alkaline phosphate
+ HLA-B27
—Not required for diagnosis
Pain greater than 3 months with age less than 45
HLB27 postive w/ >2 SpA features
Or
Sacroillitis and and >1 SpA feature
Think
Ankylosing Spondylitis
SpA features: Inflammatory Back Pain Arthritis Enthesitis Uveitis Dactylitis Psoriasis Crohns Good response to NSIADS FamHx HLBA27 Elevated CRP
What is an independent RSK fx for mortality in Ankylosing spondylitis
Cervical spine fx is an independent predictor of inpatient mortality
Tx approach to Ankylosising Spondylitis
1) NSAIDs
- very good response
2) PT
- 1st line Tx (Active>passive)
3) Steroids
- Directed at the SI joint
4) DMARDs
- Sulfasalazine/ Methotrexate
5) Biologics
- 2nd line, TNF Inhibitors -amaubs
6) Surgery
(Refer, stop smoking, Rhemo and Ortho)
HLBA 27 (Ankylosing Spondylitis) \+ Gastro S/s
Refer to
Gastro (IBD)
And Rheum
What is the common Gastro commorbidity with Arthritis
IBD
Chrons or Ulcerative Colitis
What is the approach to IBD associated Arthritis
NSAIDs avoided as first line because they may lead to exacerbations of IBD.
TNF inhibitors are recommended for active axial ankylosing spondylitis w/ concomitant IBD
Should be co-managed by rheumatology & gastroenterology
Pt that cant pee, cant see, cant bend the knee
Postinfectious Triad:
- Conjunctivitis
- Arthritis
- Urethritis
Dx for Reactive Arthritis
Develops days to weeks following GI/GU infection
Typically Monoarthritis
In a young male pt mc with chlamydia
Hallmark: Enthesitis: inflammation at sites where tendons attach to bone, MC at Achilles
This finding in a pt with reactive arthritis
Keratoderma blennorrhagica:
erythematous macules and vesicles, progressing to papules, hyperkeratotic plaques, and pustules
Classic clinical triad of Reactive Arthritis
Classic Clinical Triad
Conjunctivitis (or uveitis), Urethritis, Oligoarthritis
Pts can also present with a 4th: Mucocutaneous lesions (Apthous)
What is the lab to help R/o septic arthritis and gout for reactive arthritis
Arthrocentesis should be performed to exclude septic arthritis & gout
-Usually WBC counts of 5000-50,000
-Predominantly polymorphonuclear cells and the synovial fluid is sterile (negative Gram stain & culture)
What is the Tx approach to Reactive Arthiritis
NSAIDs: First choice for articular manifestations (ibuprofen/naproxen)
Intra articular glucocorticoids may be considered for peripheral arthritis involving few joints.
Systemic steroids have limited benefit for axial symptoms
Ophthalmology referral if uveitis is present
Antibiotics: Appropriate short term therapy if infection remains active. Does not improve the arthritic symptoms.
Consider DMARD (sulfasalazine or methotrexate) for those that fail NSAIDS and glucocorticoids
Psoriatic Arthritis on X-ray
Radiographic findings of erosions, osteolytic destruction of the interphalangeal joints, and juxta-articular new bone formation
What is a distinguishing feature of psoriatic arthritis
Enthesitis is a distinguishing feature in the pathogenesis of psoriatic arthritis
Psoriatic Arthritis is most common to what joints
Fingers (DIP) with stiffness, swelling, nail pitting
Distinguishes from RA
What are the 5 manifestations for Psoriatic Arthritis
Articular involvement
Dactylitis
(uniform swelling of the digit)
Enthesitis
Skin and nail changes
Extra articular manifestations
-HTN, T2DM, Fatty liver, Occular inflammation, IBD
What is the most common finding in psoriatic arthritis
MC finding is joint –space narrowing & erosions
Distinguished from RA by the absence of juxta-articular osteopenia & presence of pathologic new bone formation
“Pencil-in-cup” (sharpened point) appearance to DIP with Arthritis Mutilans
Periostitis in MCP, MTP, phalanges (“fluffy” appearance)
What is the treatment approach to psoriatic arthritis
Treat the s/s of inflammation
-NSAIDs (1st line
-Steroids ( rheum consult)
-DMARDS (rheum consult)
MC Methotrexate)
RA spares what joint?
DIP and typically spares the spine
When is RA worst
Morning Stiffness
- Hallmark of inflammatory arthritis and RA
- Worse in AM or after prolonged periods of rest
- Lasts for hours and improved with activity
Boutonnière deformity
(“knuckle being pushed through a buttonhole”)
flexion of the PIP joints and hyperextension of the DIP joints
Swan Neck deformity
hyperextension of the PIP joints and flexion at the DIP joints
What is the triad of Fetly Syndrome (RA)
RA
Splenomegaly
Leukopenia
(usually neutropenia, <2000 mm3)
What is the hallmark lab in RA
Anti-CCP antibodies: present 60-70%
Rheumatoid Factor: present ~50% at presentation
How do you start DMARDs for RA
Bridge tx
Takes 2-6 months to read peak effect
Typically 5-10mg prednisone daily while titrating DMARD to effect
What are the ADE of methotrexate
This is the anchor of Rx for RA
ADE: Bone marrow suppression and development of pneumonitis
Hydroxychlorquine
Usually used in conjunction with other synthetic DMARDS (i.e. methotrexate)
-Least toxic, but least effective as stand-alone tx
-Retinal toxicity—uncommon, but serious
-Increased risk over time—cumulative dose
—Initial eye exam and annual eye exams after 5 yrs of tx
Monitor: CBC, LFT, BMP, UA w/ micro-
Leflunomide
DMARDS
Comparable to Methotrexate
-Teratogenic, hepatic toxicity
Monitor: Pregnancy test, CBC, LFT, TB (baseline), hepatitis screening panel (baseline), blood pressure at baseline
Long half life—women need blood tests drawn prior to pregnancy even if stopped med years prior
Use cholestyramine to eliminate leflunomide if woman is considering to become pregnant
What are the 4 things biological DMARDs do
Inhibit Tumor Necrosis Factor
(anti TNF agents)
Deplete CD20+ B Cells
(rituximab)
Inhibit T-Cell costimulation (abatacept)
Block the receptor for interleukin-6 (tocilizumab)
What is the absolute C/I for Biological DMARDs
Active TB or Untreated latent TB is an absolute contraindication to use of biologic DMARDs
Reactivation of latent TB has occurred within weeks of starting anti-TNF agents
Tx approach to RA
RA should be diagnosed early and DMARD started at time of diagnosis
- Treatment goal of remission or low activity
- Meeting treatment goal more important than medication choice
- Most patient will need combo DMARD therapy. Typically methotrexate + something else
NSAIDS and glucocorticioids should be used sparingly as needed or a bridge therapy
DONT FORGET THESE SCREENS FOR RA tx
Consider phone consultation first to initiate therapy until patient is seen
Test for TB, Hepatitis B, C, HIV etc
Immunizations prior to immunosuppression (pneumococcal, flu)
Bridge communication between Rheumatologist and PCM
Define scleroderma
Scleroderma = Hard Skin
-systemic autoimmune disease characterized by varying degrees of skin fibrosis, vascular damage, and a wide array of internal organ dysfunction.
Two Main Manifestations
-Fibrotic dz (skin tightening & interstitial lung dz (ILD)
—Limited cutaneous (confined to fingers, toes & face)
—Diffuse cutaneous (skin tightening extends proximal to elbows, knees & trunk)
Vascular dz (Raynaud phenomenon & pulmonary arterial HTN (PAH)
What are the two prominent S/S in scleroderma
Raynuads and ANA Abs
What is the most common cause of death in scleroderma
Most common cause of death is cardiac involvement (~30%) followed by lung dz (~25%)
All require evaluation by a rheumatologist
What define diffuse scleroderma
Raynaud presentation at onset of scleroderma Dx
Skin tight ting at the proximal extremity AND TRUNCAL
More ACS, More valvular disorders, Pulm HTN group 2 and 3.
SEVERE GI DYSMOTILITY->
gut Failure
MORE MORE MORE SEVERE
What labs are specific to diffuse scleroderma
ntitopoisomerase (Scl-70),
anti-RNA polymerase III
What defines LIMITED scleroderma
Calcinosis!
Skin tightness DISTAL TO THE ELBOWS
And Reynauds SEVERAL YEARS!!! Before onset of scleroderma!
PULM HTN GROUP 1
LESS FREQUENT S/s
(Rare Myocardial Dysfuntion, rare renal crisis)
What labs are specific to LIMITED scleroderma
Anticentromere,
anti-Pm-Scl, anti-Th/To
What is CRESTS syndrome
Calcinosis,
Raynaud phenomenon, Esophageal dysmotility, Sclerodactyly, & Telangiectasis
Dx for LIMITED Scleroderma
What is the treatment approach to Raynauds
Cold avoidance
CCB
If 30 years old and new onset= Scleroderma!
Screen for ANA and nail fold capillary examination
In Sclerodrema Large pericardial effusions are associated with pulmonary arterial hypertension
Means
Poor prognosis
Pulmonary S/s in Sclerodrema
Active interstitial lung disease (ILD) occurs in pts with early diffuse scleroderma in 1st four yrs of illness
Pulmonary hypertension occurs over time ->long-standing disease
PFTs w/ Scleroderma
PFTs: 80% have restrictive pattern consistent with interstitial lung disease
High-resolution CT scanning: Interstitial fibrosis
GI manifestations of Scleroderma
Dysphagia
Severe reflux/GERD
If left untreated can lead to esophagitis, esophageal ulceration with bleeding, esophageal stricture, or Barrett esophagus
Scleroderma Renal Crisis
Occurs in only minority of pts
Incidence = 5% of scleroderma patients
More common in Diffuse Type Scleroderma(10-15%)
Abrupt onset of hypertension—malignant hypertension
-MOST DREADED complication of systemic sclerosis
10% present without hypertension and have poor outcome
- Elevated creatinine, proteinuria, hematuria
- Onset usually within 3-5 years of disease onset
What is the treatment for Sclerodermal Renal Crisis
EARLY Tx with an ACE!
Do we give Scleroderma pts get steroids?
NO!! Defer to specialist!!
Pulm fibrosis is more specific to what type of scleroderma
Diffuse!!
Money maker imaging for scleroderma
High res Chest CT
Cardiac screening for Scelroderma
Cardiac – EKG / Echo / Stress testing
Function/Ischemia
-Screen for pulmonary hypertension w/ annual transthoracic echocardiogram (TTE)
PFT f/u for scleroderma
Baseline and every 4-12 months depending on stage of disease and pt symptoms
Tx for scleroderma
Early Skin treatment:
mycophenolate mofetil (MMF) and cyclophosphamide (CYC)
(also used to treat PULM s/s)
(may progress to pulm transplant)
Treat skin pain with: gabapentin
DO NOT USE STEROIDS!
RITUXIMAB emerging Tx!!
(very promising)
Raynauds: Avoid cold and stress,
Rx: Dihydropyridine CCB
(NOT VERAPAMIL AND DILTIZAEM!)
Encourage to eat small frequent meals to control GI S/s -GERD:PPI/ H2 blockers -Delayed Gastric Emptying: Metoclopramide/ erythromycin (Gut stimulating)
Octreotide: IBS s/s (severe)
Rotating ABX for diarrhea
And PROBIOTICS FOR ALL PTS!!
(yogurt)
Maintain MOBILITY with PT
Arthritis/ Arthralgias: Methotrexate!
A pt presents with Raynauds, GERD, Skin changes, Swollen fingers/ calcifications, and arthralgias
What is the approach
Sclerodrema W/u
Negative ANA= seek other Dx
Define Chronic inflammatory disorder mainly characterized by lymphocytic infiltration of exocrine glands prominently.
Usually presents as persistent dryness of the mouth and eyes due to functional impairment of the salivary and lacrimal glands
Sjogren Syndrome
Primary vs Secondary Sjorgens Syndrome
Primary – SjS in absence of another rheumatologic disease
Secondary (also known as “associated” SjS) –
-SjS with another rheumatologic disease (usually RA, systemic sclerosis, or SLE)
What is the typical pt with sjorgerns syndrome
Primarily affects white perimenopausal women
What is the KEY feature in Sjorgrens
SICCA features: Xerostomia
Occular dryness, and eye “fatigue”
Salivary gland dysfunction and edema
What is the endocrine co-morbid with Systemic SJS dz
Thyroid Dz
What are the specific labs for primary SJS. And Secondary SJS
Primary: Anti Ro.
Secondary; Anti La
CBC findings for SJS
Normochromic, normocytic anemia
Isolated cases of hemolytic anemia
Mild leukopenia, lymphopenia, neutropenia
Mild thrombocytopenia
Hypergammaglobulinemia
Monoclonal band
=
SJS , with high incidence rate of lymphoma and cancer
Treatment for SJS
DRY eye: preservatives free art. Tears
- punctual occlusion
- topical cyclosporine (consult rheum)
Dry Mouth: sialogogues (Pilocarpine)
Lemon Drops
-meticulous oral hygiene
AVOID ETOH AND TOBACCO
AVOID ANTIHISTAMINES
Prednisoe for arthritis
Hydroxychlorquine for mild arthritis
Pathognomonic rash assoc with dermatomyositis
Heliotrope rash / Gottron sign
Most common S/s of Inflammatroy Muscle Dz
PROXIMAL muscle weakness
NOT DISTAL IN ISOLATION
What are Gattrons papules and Gattrons Sign
Gattron’s papules: raised, violaceous lesions on the extensor surfaces
MC on MCP, PIP & DIP
(Looks like psoriatic)
Gattron’s sign: erythematous rash involving extensor surfaces
Define: red or purplish discoloration of eyelids
-This may appear as hyperpigmentation in dark skinned patients
Heliotrope Rash
ONLY WITH DERMATOMYOSITIS
Major Dx criteria for Inflammatory Muscle Dz
Muslce Bx (proximal)
+ elevated muscle enzymes
(CK, Aldolase, AST/ALT/ LDH)
Abnormal EKG
And characteristic Rash
(Dermatomyositis only
What two Anitbodies are found in Inflammatory Muscle Dz
Anti-Jo 1 antibodies (20-30%)
ANA (85-95%)
What is the 1st like treatment for proximal inflammatory muscle dz
High dose prednisone, tapered down over a year
Caution : can cause steroid induced myopathy
What are the add on Rx for pts that do not recover with in 2-3 months of Tx with Predinose in Inflammatory muscle Dz.
Azathioprine
Methotrexate (caution of ILD)
Myclophenolate mofetil
Rituximab
IVIG
Cyclophosphamide
CANT LEAP Rx for GOUT
Cyclosporine Alcohol Nicotinic acid (Niacin) Thiazide diuretics Lasix (loop diuretics) Ethambutol Aspirin Pyrazinamide
Gout on US
Double contour sign
Superficial, hyperechoic band on the articular cartilage
Treatment appraoch to gout
Start with MAX dose of NSAIDS
Indomethacin (or other NSAIDs)
+ colchicine (1.2 mg at first flare) followed by a half some with in an hour
(Max dose of 1.8 mg/ hr)
Prophylaxis colchicine dose for gout
Patients receiving prophylaxis therapy may receive treatment dosing; wait 12 hours before resuming prophylaxis dose
Prophylaxis:
0.6 mg once or twice daily; maximum: 1.2 mg/day
Approach to Urate Lowering Tx for gout
One or more tophi Frequent attacks -2+ attacks a year CKD stage 2 or worse Nephrolithiasis
Goal of uric acid <6.0
-Maintaining the serum level at this target allows precipitated crystals to dissolve and be cleared
(Goal of less than 5 if tophi present)
Agent of choice: Allopurinol
In gout tx. This rx is Reserved for those with severe gout with abundant tophaceous deposits.
Pegloticase
What is the most common way to classify primary vasculitis
Vessel Size
Pt from turkey with a vascular dz
Think
behcets
What is Mono-neuritis multiplex
Foot drop or wrist drop
What is the most common vasc dz in adults
Giant cell Arteritis
Pts are over the age of 50 and more commonly female
A pt presents with HA and Jaw Claudication, scalp tenderness, with visual changes, and polymyalgia Rheumatica
Think
Giant cell Arteritis
ESR >50 (dx criteria)
Age over 50
New headache
Bx with tissue!!
Treat urgently with HIGH DOSE Steroids and admit if vision changes.
(and prophylax for j. Jevichie pneumonia)
Tx for Giant Cell
If vision changes:
Admit and IV-> PO steroids (methylpredinsone)
If no vision changes:
Prednisone
Taper Steroids down after ESR returns to normal (10% taper per week)
A pt presents with “feeling old” and muscle pain, NOT IN THE JOINT. In the neck or torso, shoulder or proximal regions of the arms, hips or proximal aspects of the thighs
ESR greater than 40.
Think
Polymyalgia Rheumatica (DDX with GIANT CELL= NO EYE INVOLVEMNT)
Treat with prednisone
What are the major prognosis complications for Giant Cell
Perm. Blindness and Thoracic Aneurysm and Aortic Dissection
A pt presents with UE Claudication, typically a woman, arms hurt when she brushes her hair, ect
Think
Takayasu’s
Look for Abnormal pulse exam / unequal blood pressures
Often involved with Aortic Regurg and Angina
HIGH ESR AND CRP
What is the treatment for Asians with Takayusu
High dose prednisone 1mg/kg/day x 1 month ->
Taper to 10mg/day over 4-6 months
Very effective but relapse is common
Addition of Methotrexate or Mycophenolate Mofetil to prednisone may provide added benefit
May be good candidates for Surgical revasc surgery
10 year survival rate 80-90%
A pt presents with HLA B51, + painful aphtous ulcers of the mouth and genitals
+erythema nodosum and vision changes
Think
Behcet
Mc to people in turkey
Treatment start with steroids and refer
A pt presents with necrotizing inflammatory vasculitius of the medium arteries that spares the venous circulation and capillaries
Insidious onset with a Hx of Hep B w/in 6 months
SPARES THE LUNGS (DDX with microscopic polyangitis )
Think Polyarteritis Nodosa
WILL BE ANCA NEGATIVE!
Test for Hep B
And Get a Bx (fibroid necrosis)
Tx with High dose steroids (+cyclophosphamide if severe)
A pt presents with severe digital ischemia without internal organ involvement
That is a chronic smoker with ulcerations
Think
Thromboangitits obliterans (buergers)
- recognition of clinical findings-digital ischemia without other organ involvement
- identification of typical pattern in angiography
- exclusion of diseases that may mimic Buerger
- confirmation that ongoing tobacco exposure is present
Tx: STOP SMOKING!
What is the 2nd MC vasculitis in childhood
Kawasakis
A pt 3-5 years old presents with Bilateral nonexudative conjunctivitis, Oral changes (cracking lips, strawberry tongue, erythema of oral & pharyngeal mucosa)
Peripheral extremity changes (erythema/edema of hands/feet, periungual desquamation)
Polymorphous rash, Cervical lymphadenopathy (least common feature)
Think
Kawasakis
Tx with IVIG quickly + aspirin
If no improvement add cyclosporines
If all tx have failed then add of Steroids
Two types of small vessel vasculitis
Immune complex vs ANCA
All with have the classic Palpable purpura
What is the MC Vasculitis in Children
Henoch-schonlein purpura
Tetrad: purpura, arthritis, glomerulonephritis, and abdominal pain is often observed.
Dx Criteria: 2/4 needed
- Palpable Purpura
- Age less than 20
- Bowel Angina
- Vessel wall granulocytes on Bx
Tx for Hosoch-Schonlein Purpura
DAPSONE!
No NSAIDs!
All pts with Cryoglobinemia will have
Postive RF factor, and palpable purpura
Assoc with multiple myeloma and Hep C!
A pt has cryoglobulins in the blood with abs to Hep C or Hep C RNA
Think
Dx of Cryoglobinemia
Treat the underlying HepC
-Ledipasvir or Ribavarin
And antivirals: RITUXIMAB
Can evolve to B cell lymphoma
A pt presents with a necrotizing vasculitis WITH an URI, lower RI and renal manifestations
Think
Granulomatosis with polyangitis
(Saddle nose deformity and ANCA positive)
Order a CT
And treat with Steroids + RITUXIMAB
(Methotrexate and steroids if limited)
All pts on prolonged steroids should get prophylaxis Rx for …
All should receive Pneumocystis jiroveci pneumonia prophylaxis
-Sulfamethoxazole-trimethoprim, Dapsone
Aka Churg-Strauss Syndorme
ASTHMA!! (RARE renal involvement)
3 phases; asthma -> blood eosinophilia-> nec vasc
Tx with Steroids
