Other Arthritides Flashcards
Other Causes of Arthritis
- Sarcoidosis
• Systemic Sclerosis
• Systemic Lupus Erythematosus (SLE) - Sjogren’s syndrome
• Lyme disease
Sarcoidosis
• Chronic systemic granulomatous disease – Multiple organ involvement
- Characterized by disseminated, noncaseating granulomas
– Suggests infection as a likely cause - Mimics rheumatologic diseases
– Fever, arthritis, uveitis, myositis, rash or neurologic deficits
Sarcoidosis
• Affects young adults in their 20’s and 30’s
• Most common in US
– 8x more common in African Americans than Caucasian Americams
– 40 cases/100,000
• Woman> men
Sarcoidosis Pathogenesis
Etiology unknown
Immune response plays a central role in pathogenesis
Sarcoidosis
• Recruit inflammatory cells
– Injury to local tissue
• Increase growth factors
– Produce fibrosis
• Hypercalcemia
– Increase absorption of calcium in intestine that results from overproduction of a metabolite of vitamin D by activated macrophages
Sarcoidosis
• Depressed cellular immunity – Lymphopenia
• Increased humoral immunity
– Autoantibody production and hypergammaglobulinemia
– (+) RF or ANA – 20-30% of patients
Sarcoid Granulomas
• Contains a central core of epitheloid cells (modified macrophages) and multi- nucleated giant cells
– Surrounded by lymphocytes, macrophages, monocytes, and fibroblasts
• Affects lungs liver, and lymph nodes
Distribution of Sarcoid Granulomas
- Lung 86%
• Liver 86%
• Lymph nodes 86%
* Spleen 63%
• Heart 20%
• Kidney 19%
• Bone marrow 17% • Pancreas 6%
Sarcoidosis Clinical Presentation
- Pulmonary symptoms (40-45%)
- Asymptomatic hilar adenopathy on chest x-ray (5-10%)
- Constitutional symptoms (25%)
- Extrathoracicinflammation(25%)
Sarcoidosis Pulmonary Symptoms
** picture-> large lymph nodes
Respiratory symptoms are the most common complaint
Dry cough
Dyspnea
Nonspecific chest pain
Hemoptysis (rare initially)
Pleural effusion (rare)
Abnormal chest x-ray >90%
Regardless of initial symptoms
Sarcoidosis
Constitutional Symptoms (25%)
- Fever
-Seen more so with hepatic involvement - Weight loss
- Fatigue
-Malaise
Sarcoidosis
• Rheumatologic manifestations
• Arthritis occurs 10 – 15% of patients
• Two patterns of joint disease
-Early onset
-Late onset
Sarcoidosis
Early onset arthritis (Most Common)
Within 6 months of disease onset
Sarcoidosis (Early)
- Erythema nodosum common (66%)
- Monoarthritis unusual
– Usually 2-6 joints
• Often begins in feet and ankles
• May spread to knees, PIPJ’s, MCPJ’s, wrists, and elbows
• Axial skeleton is spared
Sarcoidosis (Early)
- Periarticular swelling common
- Non-inflammatory effusions
• Tenosynovitis
• Heel pain
• Joint pain
– Pain greater than clinical signs of inflammation would suggest
Sarcoidosis (Early)
• Radiographic presentation
– Rarely bone or cartilage damage noted
• Duration of arthritis
– Several weeks
• May be as long a 3 months
Lofgren’s Syndrome
** erythema nodosum seen in Crohns, sarcoidosis, lofgrens
• Acute arthritis
• Erythema nodosum
• Bilateral hilar adenopathy
90% remission rate
Sarcoidosis (Late)
• 6 months after onset of disease
• Mono arthritis more common than in EARLY stage
– But usually 2 or 3 joints
• Less severe/widespread
• Transient or chronic
• Knee joint (most commonly involved)
– Followed by ankles and PIPJs
• Synovial effusions
– Mildly inflammatory
Sarcoidosis (Late)
• Chronic cutaneous sarcoidosis
• NO erythema nodosum
• Dactylitis
• Pain is not as severe
Sarcoidosis (Late)
• Radiographic changes are uncommon
– Cystic changes in middle and distal phalanx of hand
– Trabecular changes
- Honeycomb pattern
Sarcoidosis
• Other rheumatologic manifestation
– Eye disease (22%)
• Uveitis
– Myositis
– rotid gland enlargement
– Mononeuritis multiplex
– Facial nerve palsy
Sarcoidosis
- Extrathoracic manifestations
– Peripheral lymphadenopathy – 75%
– Skin involvement – 33% - macular or papular sarcoidosis
- erythema nodusom
• lupus pernio
– Hepatic granulomas – 86%
– Neurosarcoidosis- 5%
– Other manifestations
• Myocardial
• Renal involvement
Lupus Pernio
• Potentially disfiguring red to violaceous plaques and nodules
• Resemble frostbite
• Usually affects the nose, cheeks, and ears
Erythema Nodosum
• Inflammation of the fat cells under the skin (panniculitis)
• Tender,rednodules that are usually seen on both shins
Sarcoidosis
– No single finding or lab test establishes a diagnosis
– Diagnosis depends on the clinical picture
• At least 2 organ systems are affected
• Evidence of noncaseating granulomas
• Exclusion of other possible causes
Sarcoidosis
• Lab Tests
- Increase ACE levels (66%)
• Increase in serum calcium (19%) - Leukopenia (28%)
Sarcoidosis
Diagnosis
– No single finding or lab test establishes a diagnosis
– Diagnosis depends on the clinical picture
• At least 2 organ systems are affected
• Evidence of noncaseating granulomas
• Exclusion of other possible causes
Sarcoidosis
• Lab Tests
- Increase ACE levels (66%)
• Increase in serum calcium (19%) - Leukopenia (28%)
Sarcoidosis
• Imaging/Procedures
• Chest x-ray
• Transbronchial lung biopsy
• (+) Kveim-Siltzbach test (intradermal sarcoid spleen extract)
Sarcoidosis Treatment
- Asymptomatic hilar adenopathy
* No treatment
• NSAID’s
• Corticosteroids
• Immunosuppressive drugs
-Efficacy has not been established
Systemic Sclerosis
AKA Scleroderma
“Hard Skin
Systemic Sclerosis
• Multisystem disease (hallmark)
– Functional and structural abnormalities of small blood vessels
– Fibrosis of the skin and internal organs
– Inflammation
– Autoimmunity
Etiology Unknown
Potential triggers
Infectious agents,
environmental toxins, and drugs,
microchimerism
Epidemiology of Scleroderma
• Acquired, noncontageous, progressive, rare disease
– 19 cases per 1 million per year in US
• Peak age of onset 35-50 years
• Blacks are at moderately increased risk
• Female-to-male ratio 3-7:1
Systemic Sclerosis
- Diffuse scleroderma
- Limited scleroderma
- CREST syndrome
3. Localized scleroderma
Diffuse Scleroderma
• Proximal skin thickening
• Rapid onset following appearance of Raynaud’s phenomenon
• Significant vessel disease – Lung, heart, GI, kidney
• Associated with ANA and absence of aniticentromere antibody
• Poor prognosis
– 10-year survival 40-60%
Limited Scleroderma
Milder symptoms
Slower onset and progression
Mainly affects extremities distal to the elbows and/or knees
Internal organ involvement is less severe CREST Syndrome
Good prognosis
10-year survival >70%
CREST Syndrome
• Calcinosis
– Calcium deposits in skin
• Raynaud’s Phenomenon
– Spasm of blood vessel in response to cold or stress
• Esophageal dysfunction
– Acid reflux
– Decrease in motility of esophagus
• Sclerodactyly
– Thickening and tightning of skin on fingers and hands
• Telangiectasias
– Dilation of capillaries causing red marks on surface of skin
Localized Scleroderma
Morphea
-Patches of fibrotic skin and subcutaneous tissues w/o systemic disease
Linear
-Longitudinal fibrotic bands that occur predominantly on the extremities and involve the feet
Raynaud’s Phenomenon
• Vasospasticdisorder
– Associated with discoloration of the digits
• Coldtemperaturesor emotional stress triggers the attacks
Raynaud’s Phenomenon
Vasospams
Pallor & Cyanosis
Hyperemic phase
White, Blue, Red
Raynaud’s Phenomenon
• Primary syndrome
• Secondary syndrome
Primary
• Symmetric episodic attacks of acral pallor or cyanosis
• Absence of PVD
• Absence of tissue necrosis
• Normal nail-fold capillary
• Neg ANA
• Normal ESR
• Patients are usually younger
Secondary
• More severe
• Ischemicchanges/necrosis • Digitalulceration
• Associated
– Scleroderma, SLE, myositis, Sjogren’s syndrome, RA, mixed connective tissue disease
• Laterageofonset
• Asymmetric finger involvement • Abnormalnail-foldcapillary
Raynaud’s Phenomenon Treatment
- Conservative,non-pharmacologic
• Avoid exposure to cold temperatures - Avoid vasoconstrictors
– Decongestants, amphetamines, beta-blockers, caffeine, and smoking
• Calcium channel blockers – Nifedipine 30-180 mg daily – Amlodipine 5-20 mg daily
• Directvasodilator– Topical nitroglycerin
• Low-dose aspirin
Systemic Sclerosis
• Cutaneous involvement
• Diffuse pruritus
• Non-pitting edema
• Progressive skin tightness & decreased flexibility
• Varying degrees of hypo or hyperpigmentations
Systemic Sclerosis
• Subcutaneous calcinosis
• Telangiectasias
Dilated capillary loops and areas of loss of capillaries in the skin of the nail fold are characteristic
Telangiectasia
Clinical Presentation
• Musculoskeletal
– Earliest symptoms
• Nonspecific arthralgias and myaligias • Pain with motion of joints
– Secondary to inflammation and fibrosis of tendon sheath or adjacent tissue
– Late
• Atrophy and weakness
Other features
Pulmonary disease Leading cause of mortality GI dysfunction
Esophageal
Other systems are affected Cardiovascular and renal
Systemic Sclerosis
• Diagnosis
-Clinical suspicion
-No single finding or lab test establishes a diagnosis
• Lab tests
-Increased sedimentation rate
- (+) ANA in speckled or nucleolar pattern (95%)
- (+) anticentromere antibodies in about 60-90% of limited disease
-(+)Scl70
Systemic Sclerosis
• Radiographs
• Calcinosis and resorption of the distal tufts • Contractures with joint space narrowing
• Osteopenia
Systemic Sclerosis
Treatment DMARDs
MSK
NSAIDs
Corticosteroids
Skin
Topical emollients Topical steroids
Raynaud’s
Avoid cold exposure
Calcium channel blockers
Systemic Lupus Erythematosus (SLE)
Autoimmune disease multisystem involvement
Clinical manifestations of the disease are diverse
Systemic Lupus Erythematosus (SLE)
• Epidemiology
- Predominantly occurs in women
- Male approx 10%
• Peak incidence is 15-40 years of age
• More common in African Americans and Hispanic-Americans vs Caucasians
• Strong family association
Systemic Lupus Erythematosus (SLE)
Exact etiology unknown
Most common presentation – Constitutional symptoms
- Fever, fatigue and/or weight loss, lymphadenopathy, splenomegaly
– Cutaneous manifestations - Skin rash
– Articular manifestations - Arthritis and/or arthralgia
Cutaneous Manifestations of SLE
- Acute Lupus Erythematosus
• Subacute Lupus Erythematosus
* Chronic Lupus Erythematosus
Cutaneous Manifestations of SLE
• AcuteLupusErythematosus – Localalized lesions
• Hallmark “butterfly rash”
– Localized to malar region
– Confluent, macular or papular erythema on the nose and
cheeks (spares the nasolabial folds)
– Lasts days to weeks
– Generalized lesions
• Macular or maculopapular erythema occurring in a
photosensitive distribution on any area of the body
• Commonly involves palmar surfaces, dorsum of the hands,
and extensor surfaces of the fingers – Skin lesions heal w/o scarring
Cutaneous Manifestations of SLE
Subacute Lupus Erythematosus
– Nonscarring, photosensitive
– Last for weeks to months
– Back, neck, shoulders and extensor surfaces
– Some drugs can induce these lesions
– Can occur in annular form
• May be mistaken for
– Fungal rash, Lyme ds, psoriasis
Cutaneous Manifestations of SLE
• ChronicLupusErythematosus
– Photosensitive lesions that can lead to skin atrophy – Scar producing
– May persist for several months.
– Discoid Lupus Erythematosus
• Most common subtype of chronic SLE
• Term “discoid” refers to the sharply demarcated disk-shaped
appearance of the lesions
• Raised, erythematosus plaques with adherent scale that
commonly occur on the scalp, face, and neck
• Assoc with squamous cell carcinoma
Systemic Lupus Erythematosus (SLE)
Alopecia
Assoc with discoid lupus
Photosensitivity
Skin rash as a result of unusual reaction to sunlight
Mucosal lesions Mouth and nose ulcers
Systemic Lupus Erythematosus (SLE)
Musculoskeletal
Very common (90%)
Symmetric, inflammatory arthritis predominantly affecting the knees, wrists, and small joints of the hands
No erosive changes
Avascular Necrosis
Most commonly affects femoral heads, tibial plateaus, and femoral condyles
Myalgias and muscle weakness
Mild joint effusions
Systemic Lupus Erythematosus (SLE)
Jaccoud’s arthropathy
Resembles RA
Non-erosive hand
deformities Ulnar deviation Hyperflexion
Hyperextension
Reducible deformities secondary to involvement of the peri-articular tissue
Systemic Lupus Erythematosus (SLE)
• Renal
• Kidney is the most commonly affected organ
• Approximately 50% of patients develop clinically evident renal disease
• Neuropsychiatric
• Mood disorders, anxiety and psychosis • Seizures
• Headaches
• Mononeuritis
Systemic Lupus Erythematosus (SLE)
Cardiopulmonary Pericarditis
Pleurisy
Other organ systems GI
abdominal pain Ocular
cotton wool spots Hematology
Leukopenia and lymphopenia Anemia
Systemic Lupus Erythematosus (SLE)
• Diagnosis
• Clinical suspicion with support of laboratory tests
• Laboratory test
• CBC with diff, Cr, urinalysis, urine/cr ratio • Serologic tests
• ANA, anti-dsDNA, anti-Ro, anti-La, anti-Smith, anti-RNP, direct Coombs, antiphospholopid antibodies, C3, C4
Autoantibodies are present for 5-7 years before clinical onsent of SLE
American College of Rheumatology Diagnostic Criteria
Need 4 out of 11
- Malar rash
- Discoid rash
- Photosensitivity
- Oral ulcers
- Arthritis
- Serositis
- Renal disorder
- Neurologic disorder
- Hematologic disorder
- Immunologic disorder
- Antinuclear antiboidy
Systemic Lupus Erythematosus (SLE)
• Recommendations
• Avoid intense sun exposure • Yearly influenza vaccination
Systemic Lupus Erythematosus (SLE)
• Treatment • NSAID’s
• Corticosteroids
• Medrol dosepack, prednisone
• Antimalarial agents
• Hydroxychloroquine (Plaquenil) 200 mg BID • Prevents 50% of SLE flares
• Immunosuppressants
• Methotrexate, leflunomide, azathioprine, mycophenolate
mofetil, cyclophosphamide
• Others
• Rituximab, belimumab
10% of SLE patients will develop Sjogren’s Syndrome
10% of SLE patients will develop Sjogren’s Syndrome
Sjogren’s Syndrome
Debilitating autoimmune disorder
Associated with non-Hodgkins lymphoma
Sicca Complex
Xerostomia (dry mouth)
Xerophthalmia (dry eyes)
Lymphocytic infiltration of the exocrine glands
Sjogren’s Syndrome
• Insidious onset
- Occurs with any age
– Peak incidence during midlife
– Increases with age
• Affects 0.05-4.8% of population - Affects all races
• Female-to-male ratio is 9:1
Sjogren’s Syndrome
• Etiology unknown
– Possible environmental agent may trigger cascade of events
– Multiple viruses
• Predilection for causing Sjogren’s syndrome
– Hormones ???
– Genetic factors
Sjogren’s Syndrome
Primary Sjogren’s
No other underlying rheumatic disorder is present
Secondary Sjogren’s Associated
Systemic lupus erythematosus Rheumatoid arthritis
Scleroderma
Cardinal Manifestations of Sjogren’s Syndrome
• Ocularsymptoms
– Decrease aqueous component of tears
• Visual acuity and discomfort occurs
– Burning
• Relieved by artificial tears – Dryness
– Inflammation of eyelids – Foreign-body sensation – Red eye
– Painful eye
Cardinal Manifestations of Sjogren’s Syndrome
• Oral
– Dryness (xerostomia)
– Dental caries
• Gum line
– Sudden increase in salivary gland pain or size
– Enlarged
• Salivary and parotid glands
– Oral candidiasis
Other Manifestations of Sjogren’s Syndrome
• Cutaneous
– Dry skin (dec secretion of sebaceous gland)
– Vasculitis
– Periungual telangiectasis
• If in large numbers, increase chance of developing scleroderma
• Neurologic
– Peripheral neuropathy
Other Manifestations of Sjogren’s Syndrome
Musculoskeletal
• Myalgias
- Arthralgias
* Arthritis
-Symmetrical polyarthritis
-Resembles RA but nondeforming
Sjogren’s Syndrome Labs
RF 90%
ANA 80%
Sjogren’s Syndrome Treatment
• Artificial tears
• Stimulate tear production
– Restasis (cyclosporine) eye drops
• Artificial saliva
• Frequent dental care
– Flouride treatment and antimicrobial rinses
• Increases salivary secretion
– Pilocarpine
– Evoxac (cevimeline) 30 mg TID
• Antifungals
• NDAID’s
• Corticosteroids
• Immunosuppressants
Lyme Disease
Multisystem inflammatory disease
Tick-borne spirochete Borrelia burgdorferi
Lyme Disease
93 % of all Lyme disease in US
are in 11 states NY, NJ, CT, RI, MA, PA, WI, MN, MD, NH, ME
Lyme Disease
Diagnosed based on symptoms
Lyme Disease
ONLY Ixodid ticks are known to spread
B. P
Pathogenesis unclear
Increase in Incidence of Lyme Disease
Growth of deer and tick population
– Expansion of endemic areas
– People moving into endemic areas
– Increase in recognition
Clinical Presentation of Lyme Disease
Three stages (variable, w/ overlapping symptoms)
Early localized Early disseminated Late disease
Early Localized
• Erythema migrans (Hallmark)
– Present 80%
– Within a month of a tick bite – May feel warm to touch
– Rarely itching or painful
– “bulls eye”
• May expand upto12inch
• Flu-like symptoms
– Fever, chills, headache, fatigue, muscle and joint aches
• Also may have swollen lymph nodes
Early Disseminated Lyme Disease
Within weeks of onset of infection
Systems affected
Skin
Multiple erythema migrans lesions
Heart
AV block
light-headedness, fainting, shortness of breath, heart palpitations, or chest pain
1% of Lyme ds cases
Nervous systems
Cranial nerve palsy
Bell’s palsy
dysfunction of the cranial nerve VII (the facial nerve)
Meningitis
Early disseminated Lyme disease;
multiple red lesions with dusky centers
Late Disease
• Months after onset of disease
- Severe headaches and neck stiffness
- MSK
– Arthritis with severe joint pain and swelling, particularly the knees and other large joints
• Peripheral sensory neuropathy (60%)- “stocking and glove” distribution
• Reduced vibratory sensation
- “stocking and glove” distribution
Lyme Disease
Muscluloskeletal compliants reported in 80% of people with Erythema migrans
Muscluloskeletal compliants reported in 80% of people with Erythema migrans
Musculoskeletal Manifestations of Lyme Disease
• Common in all 3 stages
- Migratory pain in joint, tendons, bursae and muscle
– Pain last for hrs to few days in one location - Arthritis begins months or year after infection
– Monoarthritis or oligoarthritis
– Knee most common joint affected
Lyme Disease Diagnosis
• Based on clinical history and exposure to the tick
• Serologic tests to confirm disease
• ELISA (enzyme linked immunosorbent assay)
• Western blot
• Joint aspiration or spinal fluid
• Inflammation fluid
Immunoblot tests for Lyme disease testin – IgM and IgG
- IgM antibodies are made sooner
– Helpful for identifying patients during the first few weeks
of infection.
– Downside of testing for IgM antibodies is that they are
more likely to give false positive results. - IgG antibodies are more reliable
– Can take 4-6 weeks for the body to produce in large enough quantities for the test to detect them.
False positive results
• Tick-borne relapsing fever
• Syphilis
• Anaplasmosis(formerlyknownasgranulocytic ehrlichiosis)
• Leptospirosis
• Some autoimmune disorders(e.g.,lupus)
• Bacterial endocarditis
• Infection with Helicobacterpylori, EpsteinBarr virus, or Treponema denticola (bacteria found in the mouth that can cause gum disease and/or infection after dental procedures)
Best Treatment
Prevention
Best Treatment
Prevention
Prevent Lyme Disease
• Insect repellent
– with DEET or Permethrin
– 20 to 30% DEET (N, N-diethyl-m-toluamide
– Treat clothing and gear, such as boots, pants, socks and tents
with products containing 0.5% permethrin.
• Remove tick promptly
– Conduct a full-body tick check using a hand-held or full-length mirror to view all parts of your body upon return from tick- infested areas.
• Apply pesticides
• Reduce tick habitat
– Clear tall grasses and brush around homes and at the edge of lawns.
– Place a 3-ft wide barrier of wood chips or gravel between lawns and wooded areas and around patios and play equipment. This will restrict tick migration into recreational areas.
Removing a tick
• Usefine-tippedtweezerstograspthetickas close to the skin’s surface as possible
• Pull upward with steady, even pressure.
Don’t twist or jerk the tick; this can cause the mouth- parts to break off and remain in the skin. If this happens, remove the mouth-parts with tweezers. After removing the tick, thoroughly clean the bite area and your hands with rubbing alcohol, an iodine scrub, or soap and water
• Dispose of alive tick by submersing it in alcohol, placing it in a sealed bag/container, wrapping it tightly in tape, or flushing it down the toilet. Never crush a tick with your fingers
If you remove a tick quickly (within 24 hours), you can greatly reduce your chances of getting Lyme disease.
If you remove a tick quickly (within 24 hours), you can greatly reduce your chances of getting Lyme disease.
Prevention
• Most humans are infected through the bites of immature ticks called nymphs
– Nymphs are tiny (less than 2 mm) and difficult to see
– They feed during the spring and summer months
• Adult ticks can also transmit Lyme disease
bacteria
– Much larger and are more likely to be discovered and removed before they have had time to transmit the bacteria
– Adult Ixodes ticks are most active during the cooler months of the year
Prevent Lyme Disease
• Insect repellent
– with DEET or Permethrin
– 20 to 30% DEET (N, N-diethyl-m-toluamide
– Treat clothing and gear, such as boots, pants, socks and tents
with products containing 0.5% permethrin.
• Remove tick promptly
– Conduct a full-body tick check using a hand-held or full-length mirror to view all parts of your body upon return from tick- infested areas.
• Apply pesticides
• Reduce tick habitat
– Clear tall grasses and brush around homes and at the edge of lawns.
– Place a 3-ft wide barrier of wood chips or gravel between lawns and wooded areas and around patios and play equipment. This will restrict tick migration into recreational areas.
Treatment of Lyme Disease
• Early localized
– Oral Antibiotic treatment (2-3 wks)
• Curative
• Doxycycline, amoxicillin, cefuroxime
• Early disseminated and Late
– IV antibiotics (2-4 wks)
• Cefotaxime • Penicillin G
