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Flashcards in Other Arthritides Deck (101):
1

Other Causes of Arthritis


• Sarcoidosis
• Systemic Sclerosis
• Systemic Lupus Erythematosus (SLE)
• Sjogren’s syndrome
• Lyme disease

2

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

3

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

4

Sarcoidosis Pathogenesis


Etiology unknown
Immune response plays a central role in pathogenesis

5

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

6

Sarcoidosis


• Depressed cellular immunity – Lymphopenia

• Increased humoral immunity

– Autoantibody production and hypergammaglobulinemia
– (+) RF or ANA – 20-30% of patients

7

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

8

Distribution of Sarcoid Granulomas


• Lung 86%
• Liver 86%
• Lymph nodes 86%
• Spleen 63%
• Heart 20%
• Kidney 19%
• Bone marrow 17% • Pancreas 6%

9

Sarcoidosis Clinical Presentation


1. Pulmonary symptoms (40-45%)
2. Asymptomatic hilar adenopathy on chest x-ray (5-10%)
3. Constitutional symptoms (25%)
4. Extrathoracicinflammation(25%)

10

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)

11

Abnormal chest x-ray >90%


Regardless of initial symptoms

12

Sarcoidosis


Constitutional Symptoms (25%)


-Fever
-Seen more so with hepatic involvement
-Weight loss
-Fatigue
-Malaise

13

Sarcoidosis

• Rheumatologic manifestations



• Arthritis occurs 10 – 15% of patients
• Two patterns of joint disease
-Early onset
-Late onset

14

Sarcoidosis

Early onset arthritis (Most Common)


Within 6 months of disease onset

15

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

16

Sarcoidosis (Early)

• Periarticular swelling common
• Non-inflammatory effusions
• Tenosynovitis
• Heel pain
• Joint pain
– Pain greater than clinical signs of inflammation would suggest

17

Sarcoidosis (Early)

• Radiographic presentation
– Rarely bone or cartilage damage noted
• Duration of arthritis
– Several weeks
• May be as long a 3 months

18

Lofgren’s Syndrome


** erythema nodosum seen in Crohns, sarcoidosis, lofgrens

• Acute arthritis
• Erythema nodosum
• Bilateral hilar adenopathy

90% remission rate

19

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

20

Sarcoidosis (Late)


• Chronic cutaneous sarcoidosis


• NO erythema nodosum
• Dactylitis
• Pain is not as severe

21

Sarcoidosis (Late)


• Radiographic changes are uncommon

– Cystic changes in middle and distal phalanx of hand
– Trabecular changes
- Honeycomb pattern

22

Sarcoidosis

• Other rheumatologic manifestation

– Eye disease (22%)
• Uveitis

– Myositis
– rotid gland enlargement
– Mononeuritis multiplex
– Facial nerve palsy

23

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

24

Lupus Pernio

• Potentially disfiguring red to violaceous plaques and nodules
• Resemble frostbite
• Usually affects the nose, cheeks, and ears

25

Erythema Nodosum


• Inflammation of the fat cells under the skin (panniculitis)
• Tender,rednodules that are usually seen on both shins

26

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

27

Sarcoidosis

• Lab Tests



• Increase ACE levels (66%)
• Increase in serum calcium (19%)
• Leukopenia (28%)

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

29

Sarcoidosis
• Lab Tests


• Increase ACE levels (66%)
• Increase in serum calcium (19%)
• Leukopenia (28%)

30

Sarcoidosis
• Imaging/Procedures


• Chest x-ray
• Transbronchial lung biopsy
• (+) Kveim-Siltzbach test (intradermal sarcoid spleen extract)

31

Sarcoidosis Treatment


• Asymptomatic hilar adenopathy
• No treatment
• NSAID’s
• Corticosteroids
• Immunosuppressive drugs
-Efficacy has not been established

32

Systemic Sclerosis


AKA Scleroderma
“Hard Skin

33

Systemic Sclerosis


• Multisystem disease (hallmark)
– Functional and structural abnormalities of small blood vessels
– Fibrosis of the skin and internal organs
– Inflammation
– Autoimmunity

34

Etiology Unknown

Potential triggers
Infectious agents,
environmental toxins, and drugs,
microchimerism

35

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

36

Systemic Sclerosis


1. Diffuse scleroderma
2. Limited scleroderma
-CREST syndrome
3. Localized scleroderma

37

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%

38

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%

39

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

40

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

41

Raynaud’s Phenomenon


• Vasospasticdisorder
– Associated with discoloration of the digits

• Coldtemperaturesor emotional stress triggers the attacks

42

Raynaud’s Phenomenon


Vasospams
Pallor & Cyanosis
Hyperemic phase
White, Blue, Red

43

Raynaud’s Phenomenon


• Primary syndrome
• Secondary syndrome

44

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

45

Secondary


• More severe
• Ischemicchanges/necrosis • Digitalulceration
• Associated
– Scleroderma, SLE, myositis, Sjogren’s syndrome, RA, mixed connective tissue disease
• Laterageofonset
• Asymmetric finger involvement • Abnormalnail-foldcapillary

46

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

47

Systemic Sclerosis


• Cutaneous involvement
• Diffuse pruritus
• Non-pitting edema
• Progressive skin tightness & decreased flexibility
• Varying degrees of hypo or hyperpigmentations

48

Systemic Sclerosis


• Subcutaneous calcinosis
• Telangiectasias
Dilated capillary loops and areas of loss of capillaries in the skin of the nail fold are characteristic

49

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

50

Other features


Pulmonary disease Leading cause of mortality GI dysfunction
 Esophageal
Other systems are affected Cardiovascular and renal

51

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

52

Systemic Sclerosis

• Radiographs



• Calcinosis and resorption of the distal tufts • Contractures with joint space narrowing
• Osteopenia

53

Systemic Sclerosis

 Treatment  DMARDs


 MSK
 NSAIDs
 Corticosteroids
 Skin
 Topical emollients  Topical steroids
 Raynaud’s
 Avoid cold exposure
 Calcium channel blockers

54

Systemic Lupus Erythematosus (SLE)

Autoimmune disease multisystem involvement
Clinical manifestations of the disease are diverse

55

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

56

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

57

Cutaneous Manifestations of SLE


• Acute Lupus Erythematosus
• Subacute Lupus Erythematosus
• Chronic Lupus Erythematosus

58

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

59

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

60

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

61

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

62

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

63

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

64

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

65

Systemic Lupus Erythematosus (SLE)
 Cardiopulmonary  Pericarditis


 Pleurisy
 Other organ systems GI
 abdominal pain  Ocular
 cotton wool spots  Hematology
 Leukopenia and lymphopenia  Anemia

66

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

67

American College of Rheumatology Diagnostic Criteria
Need 4 out of 11


1. Malar rash
2. Discoid rash
3. Photosensitivity
4. Oral ulcers
5. Arthritis
6. Serositis
7. Renal disorder
8. Neurologic disorder
9. Hematologic disorder
10. Immunologic disorder
11. Antinuclear antiboidy

68

Systemic Lupus Erythematosus (SLE)
• Recommendations


• Avoid intense sun exposure • Yearly influenza vaccination

69

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

70

10% of SLE patients will develop Sjogren’s Syndrome

10% of SLE patients will develop Sjogren’s Syndrome

71

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

72

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

73

Sjogren’s Syndrome
• Etiology unknown


– Possible environmental agent may trigger cascade of events
– Multiple viruses
• Predilection for causing Sjogren’s syndrome
– Hormones ???
– Genetic factors

74

Sjogren’s Syndrome

Primary Sjogren’s


No other underlying rheumatic disorder is present
Secondary Sjogren’s  Associated
Systemic lupus erythematosus Rheumatoid arthritis
 Scleroderma

75

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

76

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

77

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

78

Other Manifestations of Sjogren’s Syndrome

Musculoskeletal
• Myalgias


• Arthralgias
• Arthritis
-Symmetrical polyarthritis
-Resembles RA but nondeforming

79

Sjogren’s Syndrome Labs


RF 90%
ANA 80%

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

81

Lyme Disease

Multisystem inflammatory disease
Tick-borne spirochete Borrelia burgdorferi

82

Lyme Disease

93 % of all Lyme disease in US



are in 11 states NY, NJ, CT, RI, MA, PA, WI, MN, MD, NH, ME

83

Lyme Disease

Diagnosed based on symptoms

Lyme Disease
ONLY Ixodid ticks are known to spread
B. P

Pathogenesis unclear

84

Increase in Incidence of Lyme Disease


Growth of deer and tick population
– Expansion of endemic areas
– People moving into endemic areas
– Increase in recognition

85

Clinical Presentation of Lyme Disease

Three stages (variable, w/ overlapping symptoms)



Early localized Early disseminated Late disease

86

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

87

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

88

Early disseminated Lyme disease;

multiple red lesions with dusky centers

89

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

90

Lyme Disease

Muscluloskeletal compliants reported in 80% of people with Erythema migrans


Muscluloskeletal compliants reported in 80% of people with Erythema migrans

91

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

92

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

93

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.

94

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)

95

Best Treatment

Prevention

Best Treatment

Prevention

96

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.

97

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

98

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.

99

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

100

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.

101

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