1. Connective Tissue Diseases
Sjogren’s Syndrome
Idiopathic Inflammatory
Myopathies IIM, Scleroderma

2. • A 52-year old female grade-school
teacher presents with a two-year history
of extreme fatigue, and oral dryness. She
has noticed increased difficulty getting
through a full day at school due to
muscle/joint pain, fatigue, and difficulty
speaking. She is menopausal. You ask
about dryness of the eyes and she admits
to frequent use (4 times per day) of
artificial tears and photosensitivity. She
describes some type of rash when she
goes into the sun.

3. • The oral exam is significant for
mucosal dessication, fissured
tongue, and dental decay. She
exhibits muscle trigger point
tenderness on the back of the
neck, shoulder, elbows, knees,
lower back and hips. She
complains of joint pain in the
knees and hands. No signs of
vasculitis, skin rash, or joint
swelling are observed.

4. Which of the following should be
included in the differential
diagnosis? ?
• Fibromyalgia
• Primary Sjogren’s syndrome
• SLE
• Secondary SS with SLE
• Secondary SS with RA
• All of the above

5. Sjogrens syndrome
• A chronic, systemic, autoimmune,
inflammatory disorder with lymphocytic
infiltration , destruction of exocrine glands
(lacrimal , salivary) and production of
autoantibodies.
• Xerostomia -dry mouth
• Keratoconjunctivitis sicca- dry eyes
• Also affects: lungs(pneumonitis), kidneys
( instertitial nephritis), heart, skin (vasculitis),
nervous system, hematopoietic system.

6. Epidemiology
• Incidence 4/ 100 000
• F / M – 9:1
• Onset 35- 50 years of age
• Genetic predisposition
• Family clustering

7. SS
• Primary SS
– K.Sicca – aqueous tear deficiency
– Xerostomia – hyposalivation
• Secondary SS
– Primary SS + another Rheumatological
condition
• RA, SLE, Ssc

8. Clinical course SS
• Slowly progressive
• Risk of developing Lymphoma 3-5% in life
time in Primary SS
• Mortality ratio for PSS similar to normal
population

9. Systemic involvement
• Cutaneous – dryness and pruritus
• Vasculitis –
– palpable or non palpable purpura of the lower
extremities
– In crops and may ulcerate
– Urticarial vasculitis
– Nodular vasculitis

10. Systemic involvement
• Upper airway ds
– Recurrent non-allergic rhinitis / sinusitis
– Dry cough
– Bronchial dryness – mucus plugging
• Lung Disease
– ILD – most common
– Bibasilar crepitation, before cough/DOE

11. Systemic involvement
• Heart Ds
• Echocardiographic evidence of past
pericarditis
• Hypokinesia LV
• Congenital heart block in infants and adults
related to anti – SSA

12. Systemic involvement
• GI and Hepatic
– Disphagia
– Esophageal dysmotility
– Nausea, epigastric pain, dyspepsia
– Primary biliary cirrhosis

13. Systemic involvement
• Renal Disease
– Instertitial nephritis
• Dysuria
• Urinary frequency
• Nocturia
• And urgency in the absence of infection
– Mild proteinuria

14. Systemic involvement
• Genitourinary
– Dyspareunia
– Interstitial cystitis
• Neuropathies
– Peripheral – “glove and stocking”
• Fatigue
• Endocrine disorders – thyroid ds

15. Other clinical features
• Non – erosive arthritis/arthralgias
• Raynaud’s
• Lymphadenopathy
• Vasculitis
• Myositis

16. Criteria for the classification of
Sjögren’s syndrome
• Ocular Symptoms
• Oral Symptoms
• Ocular Signs
• Schirmer test < 5 mm
• Rose Bengal score ³ 4
• Histopathology ³ 1 agglomeration of 50 or more mononuclear cells/4mm
• tissue (focus score)
• Objective evidence of salivary gland involvement
• Autoantibodies
• SSA/Ro, SSB/La, ANA, RF
• (4 or > high sensitivity and specificity)
• Exclusions: lymphoma, sarcoid, GVH, acquired immune deficiency

17. Sjögren’s syndrome: evaluation
• Schirmer test – measures tear production via mm of wetness
• Rose Bengal or fluorescein stain – detects disruption or devitalized tissue
• Salivary flow – amount of saliva produced
• Dental evaluation
• Minor salivary gland (lip) biopsy – looking for lymphoid infiltration
• Serologic tests (SSA(Ro), SSB(La), ANA, RF)
• SPEP, cryoglobulins
• Lymph node biopsy
• Evaluation for renal tubular acidosis

18. SS – diff dx oral dryness
• Drugs
• Tricyclics antidepressants
• Cold remedies
• Antihystamines
• Diuretics antihypertensives
• Anti – cholinergics
• Acute anxiety or depression
• Mouth breathing
• Autoimmune Ds
• Viral cond. HIV, Hep C
• Central brain lesions
– Alzheimer’s, Multiple Sclerosis
• Head and Neck radiation
• Congenital absence of salivary glands

19. Questions-hyposalivation
• Do you have to drink water at night?
• Do you keep water at bedside?
• Can you swallow a cracker w/o water?
• Do you become easily choked?
• Recent increased dental decay?
• Change in the way food taste ?
• Yes response – support hyposalivation

20. DDx for SS
• SLE
• Sarcoidosis
• Fibromyalgia
• Fatigue and depression
• Other causes for parotid gland enlargement

21. Diagnostic procedures
• H&P
• Lab studies
– CBC, BUN, Creatinine, LFT’s, Hep C, HIV, RF,
ANA, anti Ro/SSA, anti La/SSB
– Sm RNP
– Immunoglobulins, UA
• Chest Xray
• Rose bengal, Schirmer test, Salivary flow rate
by Tech 99 uptake, MRI
• Labial salivary gland biopsy

22. Treatment for SS
• First Goal symptomatic
• Minimize serious outcomes
• Lung transplant
• Blindness
• Loss of teeth
• Depression and
• Disability

23. Pharmacological Management
• Ocular dryness
• OTC eye drops – Systane and Refersh tears
• Rx: Cyclosporine eye emulsion 0.05%
(Restasis)

24. Pharmacological Management
• Oral Dryness
– OTC: Sugar free chewing gum, and lozenges
– Rx:
• Evoxac (Cevimeline) TID
• Salagen (Pilocaroine) 3-4 time per day
(Both muscarinic cholinergic agonists)
– Avoid in patients with angina, heart block,
glaucoma, severe asthma
– Side effects: sweating, flushing , polyuria, visual
blurring and decreased nigh vision

25. Oral health
• To treat Xerostomia, Candidiasis, dental
and periodontal problems
• Oral hygiene, tooth brusing, , flossing,
dental prophylaxis every 4 months, fluoride
applications
• Artificial saliva
• Avoid drugs that worsen dry mouth

26. Oral Candidiasis Treatment
• Mycelex (Clotrimazole 10 mg lozenges)
• suck on one 4-5 times per day
• Fluconazole 100mg
– Two tables PO on day one , then one per day
for two weeks

27. Systemic therapies
• Plaquenil (Hydroxychloroquine)
• Arthralgias, skin manif. and fatigue
• Methotrexate
• Prednisone
• Rituximab

28. Sjögren’s syndrome: parotid
gland

29. Sjögren’s syndrome: B-cell
lymphoma, parotid gland
(clinical and photomicrograph )

30. Sjögren’s syndrome: cornea
(Rose Bengal stain)

31. Sjögren’s syndrome: Schirmer
test

32. Sjögren’s syndrome: xerostomia

33. Sjögren’s syndrome: parotid
gland (photomicrograph)

34. Sjögren’s syndrome: parotid
gland (sialograms)

35. • CASE # 2
• 57 year old white female presents to the
primary care clinic to establish care. She
has noticed thickening of the skin on her
hands that she initially attributed to an
allergy to dishwashing detergent.
However, she has become increasingly
concerned since it has traveled up to her
elbows bilaterally and started involving
her feet and shins. She has difficulty
making a fist due to the skin thickening.

36. • Additionally, she has been noticing
weakness doing simple tasks like
getting up off a chair. Physical exam
reveals sclerodactyly extending up to
the elbows on the upper extremity and
up to the mid-shins on the lower
extremity. There is evidence of muscle
wasting over the biceps and quadriceps
with 3/5 muscle strength of the hip
flexors. Initial laboratory evaluation was
unremarkable except for an elevated
CPK of 10,000.

37. What is the diagnosis?
• Limited Scleroderma
• Diffuse Scleroderma
• Scleroderma/myopathy Overlap
• Polymyositis

38. • The patient in this case presents with skin
involvement suggestive of limited
scleroderma. However, the proximal muscle
weakness with elevated CPK is suggestive
of muscle inflammation or myopathy.
Muscle encasement due to sclerodermatous
involvement of the fascia can cause
elevated CPK and weakness as can a
concomitant inflammation of the muscle
itself. There is no data in the case to make
the definitive diagnosis of polymyositis such
as a muscle biopsy or antibodies therefore
the answer is:

39. • C, scleroderma with some form of
myopathy. While this disease
conglomerate can be seen in
conjunction with several other
autoimmune diseases including SLE,
nothing in this patient presentation
seems to suggest a diagnosis of SLE.
Classical skin manifestations of SLE
are malar/discoid facial rash,
alopecia and oral/nasal ulcers, which
this patient does not have.

40. Subsets of Systemic Sclerosis
• Diffuse cutaneous syst.sclerosis
• Limited cutaneous syst.sclerosis
• Overlap syndromes
• Diffuse or limited with features of other CTD
• Mixed connective tissue disease MCTD
• Localized sclerodrema
• Morphea
• Linear scleroderma

41. Scleroderma
• Systemic sclerosis
– With diffuse scleroderma: rapidly progressive skin
thickening (proximal to elbows and knees), early
visceral disease (lung, heart and kidney)
– With limited scleroderma: restricted and non
progressive skin thickening (distal extremities), delay
visceral involvement (CREST)
– With overlap: diffuse or limited with features of other
CTD (PM, DM, SLE)

42. SSc Pathogenesis
• Susceptible host
• Triggering event
• Activation immune system
• Endothelial cell activation
• Activation fibroblasts
• Obliterative vasculopathy and Fibrosis
(increased collagen deposition)

43. SSc Epidemiology
• Incidence 15-20 cases per million
• Females predominant F:M – 5:1
• Age of onset 30-50 years of age
• More severe in African American

44. Scleroderma
• DCSS-
– proximal and distal skin thickening involves
face / neck and trunk
– Symmetric involvement fingers, hands, arms
and legs
– Rapid onset after Raynauds
– Auto-Ab present
– Overall prognosis poor

45. Scleroderma
• LCSS:
– CREST
– Limited to symmetrical changes on
fingers(sclerodactyly), distal arms and face and
neck
– Later visceral disease
– Abs present
– Good prognosis

46. Scleroderma Cutaneous
Manifest.
• Skin thickening
• Telangectasias
• Digital pitting scars
• Calcium deposition
• Skin ulceration

47. Other clinical manifestations
• Musculoskeletal
– Arthralgias and myalgias
– Synovitis, tendonitis
• GI
– Small oral aperture
– Esophageal dysfunction
– Bowel dysmotility

48. Cont.
• Pulmonary:
– Fibrosis and inflammation with ILD
• Cardiac
– Myocarditis, pulmonary HTN, arrhythmias
• Renal
– Scleroderma renal crisis, renal failure

49. ACR systemic sclerosis:
preliminary classification criteria
• Major criterion or
• two minor criteria for diagnosis
• Major criterion
• Proximal scleroderma
• Minor criteria
• Sclerodactyly
• Digital pitting or scars or
• loss of substance from finger pad
• Bibasilar pulmonary fibrosis

50. Scleroderma-like syndromes
• Toxin- or drug-induced scleroderma
– Organic solvents and epoxy resins
– Eosinophilic myalgia syndrome (L-tryptophan)
– Bleomycin
• Vibration injury
• Scleromyxedema
• Eosinophilic fasciitis
• Graft-versus-host disease

51. Raynaud’s phenomenon
• Episodic, reversible digital skin color change
– white to blue to red
– well-demarcated
• Due to vasospasm
• Usually cold-induced
• Primary (Raynaud’s disease) and secondary forms

52. Causes of secondary Raynaud’s
phenomenon
• Connective tissue diseases
– Scleroderma, systemic lupus erythematosus, MCTD, undifferentiated CTD,
Sjogren’s syndrome, dermatomyositis
• Occlusive arterial disease
– Atherosclerosis, anti-phospholipid antibody syndrome, Buerger’s disease
• Vascular injury
– Frostbite, vibratory trauma
• Drugs and toxins
– Beta blockers, vinyl chloride, bleomycin, ergot, amphetamines, cocaine
• Hyperviscosity/cold-reacting proteins
– Paraproteinemia, polycythemia, cryoglobulinemia, cryofibrinogenemia, cold
agglutinins

53. Raynaud’s phenomenon: hands

54. Scleroderma: Raynaud’s
phenomenon, cyanosis of the
hands

55. Scleroderma: skin induration,
hands

56. Scleroderma: acrosclerosis

57. Scleroderma: acrosclerosis and
terminal digit resorption

58. CREST syndrome: calcinosis
cutis, fingers

59. Scleroderma: calcinosis, hands

60. Scleroderma: leg ulcer

61. Scleroderma: facial changes,
lateral view

62. Scleroderma: Mauskopf, facial
changes

63. Scleroderma: Mauskopf, facial
changes

64. Linear scleroderma: en coup de
sabre, scalp and forehead

65. Linear scleroderma: thigh and leg

66. Morphea: leg

67. Scleroderma: Raynaud’s
phenomenon, hand (arteriogram)

68. Raynaud’s phenomenon: hand
(angiogram)

69. Scleroderma: acrolysis
(radiographs)

70. Scleroderma: calcinosis and
acrolysis (radiograph)

71. CREST syndrome: arm (radiograph)

72. Scleroderma: pulmonary fibrosis
(radiograph)

73. Scleroderma: wide-mouthed
diverticula, colon (radiograph)

74. Scleroderma: kidney
(arteriograms)

75. Scleroderma
• Rare connective tissue disease that has
– Fibrosis
– Vascular instability (intimal proliferation and
Raynaud’s)
– Autoimmunity

76. Is it autoimmune?
• In some patients with scleroderma the ANA
is positive
• Greater than 80% in limited scleroderma
and 50% in diffuse scleroderma

77. CREST
• Old fashioned term but still used
– Calcinosis
– Raynaud’s
– Esophageal dysmotility
– Sclerodactyly
– Telangiectasia

78. Limited vs Diffuse Scleroderma
Limited Diffuse
• Most positive ANA • 50% ANA positive
• 80% anticentromere usually nucleolar
• Rare renal, heart, lung • Scl 70 in 30%,
involvement correlates with
• May develop PAH in pulmonary fibrosis
long standing disease • Renal crisis with RNA
polymerase
• Higher mortality

79. Sclerodactyly and pigmentation

80. Raynaud’s
• Primary
– Not associated with any other disease
Secondary
Associated with connective tissue diseases such as
SLE, scleroderma, RA, Sjogren’s, Polymyositis
Reversible color change of the digitals with pallor and
then rubor and or cyanosis

81. Treatment: Raynaud’s
• Calcium channel blockers
• Cold avoidance
• Smoking cessation
• Other drugs

82. Proof of Treatment in Raynaud’s
associated with Scleroderma
• Calcium channel blockers, esp Nifedipine
– Small trials, can’t prove effectiveness for
healing of digital ulcers
Meta-analysis Arthritis Rheum 2001 44:1841-7

83. Prostacyclins/ Prostaglandin
analogues
• Iloprost
• Very effective in IV(5 trials), less effective po (1
trial)
• Effective in RP frequency and severity of attacks
and at healing and preventing digital ulcers
• Beraprost - effective for recurrent digital ulcers
J Rheumatol 1999, 26:2173-8

84. Treatment: GI Tract
• Proton pump inhibitors are very effective
for GERD
• Anti-reflux maneuvers include: not eating
after supper, raising the head of the bed,
pro-kinetic drugs to propel food through
the stomach
• Small bowel overgrowth can be treated by
antibiotics on an intermittent basis
• Some drugs can help the bowels contract

85. Incontinence can also occur secondary to:
• Hypotonic bowel
• Poor anal sphincter tone
Diverticulosis can also occur in the bowel

86. Renal Involvement
• Renal crisis is a condition with high blood
pressure (usually), hemolysis (intra-
vascular), and worsening renal function
• Renal crisis is secondary to poor blood flow
to the kidney, as well as kidney changes
with scarring around the blood vessels

87. Renal Involvement (cont’d)
• Treatment has improved the mortality from
scleroderma renal crisis, particularly rapid
control of the blood pressure using ACE
inhibitors
• Some patients do go on to temporary or
permanent dialysis

88. Lung Involvement
Two main types:
• Interstitial lung disease (inflammation and
scarring of the lung parenchyma)
• Pulmonary hypertension with high
pressures in the arteries perfusing the lungs

89. Prevalence in Scleroderma
Related PAH
• Pulmonary Hypertension (PAH) in
scleroderma is either
– Primary (vascular defect)
– Secondary (Secondary to pulmonary fibrosis)
– Or both

90. Treatment of PAH
• Same as that for Primary Pulmonary HTN
(PPH)
Vasodilators such as calcium channel blockers
Endothelin receptor antagonist (Bosentan)
Prostacyclin analogs: Epoprostenol (Flolan),
Iloprost
Anticoagulation
Treatment of CHF and dysrythmia
Oxygen
Nitric Oxide

91. Bosentan
• Bosentan (Tracleer) is an Endothelin-1
antagonist
– Endothelin-1 is a potent vasoconstrictor and
smooth muscle mitogen

92. Heart Involvement
• Patients with scleroderma can develop a
cardiomyopathy with thickening heart
muscles and reduced blood flow to the heart
• This is manifest by shortness of breath,
angina or congestive heart failure
• It is treated the same way as congestive
heart failure from other causes

93. Pleural and Pericardial Effusions
• These can occur in scleroderma,
particularly in those with diffuse
scleroderma
• Sometimes treated with prednisone

94. Arthritis in Scleroderma
• Many patients with scleroderma have
arthralgia
• Some have inflammatory arthritis with
swollen joints
• 20% on x-ray can have joint destruction
• This is treated with anti-inflammatories,
physiotherapy, and sometimes disease-
modifying drugs

95. Calcinosis
• Calcium deposits are under the skin in
pressure areas
• They can break open and ooze white,
chalky material
• They are often painful

97. Digital Tuft Resorption
• Patients with scleroderma can lose mass at
their fingertips making them painful and
appearing tapered or shortened

98. Digital Ulcers
• Digital ulcers occur in many patients with
scleroderma
• They are painful
• May take a long time to heal sometimes resulting
in gangrene or rarely necessitating amputation
• Treatment with analgesics, blood thinners, and
new drugs (ie. Bosentan) are being studied for
increased healing and a decrease in new ulcers

99. Digital Ulcers

100. other treatments are underway,
including:
• Biologic drugs, such as antibodies to decrease
TGF-beta (tumor growth factor beta), which is
important in causing fibrosis in scleroderma
• Other biologic trials are being considered for
patients, such as blocking cTGF, this is important
in fibrosis and fibroblast production in
scleroderma, and is also an important target

101. Targeted Therapies
• Anti TGFbeta antibodies – under
development, 1st trial negative
• cTGF antibodies
• Targeting pathological pathways
• Stem cell transplant study

102. Scleroderma Mortality
• Similar to breast cancer (50% 5 year survival)
• From:
– Interstitial lung disease
– Cardiomyopathy
– Pulmonary Hypertension
– “We have found that those with renal involvement still
have a very high mortality and was the highest
association of mortality in our cohort”

103. Conclusions
• Scleroderma is a rare connective tissue disease
• It is accompanied by a lot of morbidity and at
times mortality
• There are good treatments for symptom control of
various organ systems
• There are some good treatments for reversing the
progression of the organ-specific disease, such as
scleroderma renal crisis
• The future appears promising for direct targets
that may help in the treatment of scleroderma

104. Idiopathic Inflammatory
Myopathies
IIM

105. Idiopathic Inflammatory
Myopathies (IIM)
• Adult polymyositis (PM)
• Adult Dermatomyositis (DM)
• Juvenile myositis (JDMS)
• Malignancy-associated myositis
• Myositis overlap with another
rheumatic disease
• Inclusion body myositis (IBM)

106. IIM
• heterogeneous group of
autoimmune disorders
characterized by muscle weakness,
inflammation and possible
systemic complications.

107. IIM Epidemiology
• Rare disease
• Incidence 5-10 cases/ million
• Prevalence 50-90 cases /million
• F:M 2-3: 1
• African American women more affected

108. IMM Clinical
• Proximal and symmetric muscle weakness
• Functional deficit from weakness
• Difficulty raising their arms, combing the hair, getting up
from the chair, walking up steps, frequent falls
• Fatigue, joint pain , anorexia
• Elevated muscle enzymes:
• CK, AST, ALT, aldolase, and LDH
• EMG abnormal
• Abnormal muscle biopsy

109. Proposed diagnostic criteria for
polymyositis and dermatomyositis
• PM diagnosed as definite with 4 out of 5 of the below criteria or
probable with 3 out of 5
• DM diagnosed as definite with rash plus 3 out of 4 of the below
criteria or probable with rash plus 2 out of 4 criteria
– Symmetric proximal muscle weakness
– Elevated muscle enzymes (CPK, aldolase, transaminases,
LDH)
– Myopathic EMG abnormalities
– Typical changes on muscle biopsy
– Typical rash of dermatomyositis

110. Polymyositis: differential
diagnosis
• Polymyositis and dermatomyositis
• Hypothyroidism
• Drug-induced myopathies
• Corticosteroids, colchicine, HMG-CoA reductase inhibitors,
zidovudine, hydroxychloroquine, alcohol
• Infections
– Viral, toxoplasmosis, trichinosis, bacterial pyomyositis
• Connective tissue disorders
– Lupus, scleroderma, MCTD
• Systemic vasculitis
– PAN, Wegener’s granulomatosis

111. Polymyositis: differential
diagnosis, cont’d
• Metabolic myopathies
– Disorders of carbohydrate and lipid metabolism
• Electrolyte disturbances
– Hypernatremia, hyponatremia, hypokalemia, hypophosphatemia,
– hypocalcemia
• Inclusion body myositis
• Sarcoid myopathy
• Amyloid myopathy
• Neurologic disorders
– Myasthenia gravis, motor neuron disease, muscular dystrophy
•

112. Inclusion body myositis
• Males affected more than females
• Age of onset usually greater than 50
• Slowly progressive
• Distal and asymmetric muscle weakness
• Myopathic and neuropathic changes on EMG
• Mononuclear cell infiltrates and vacuoles containing amyloid on
• muscle biopsy
• Responds poorly to corticosteroids

113. Myositis-specific antibodies
ANTIBODY DISEASE ASSOCIATION PREVALENCE
Anti-tRNA Dermatomyositis, 20%
synthetases (Jo-1) interstitial lung disease,
“mechanic’s hands”
Anti-SRP (signal African-American women, Rare
recognition protein) poor prognosis
Anti-Mi-2 Older women, “shawl 5%
sign,” good prognosis
PM/SCL Polymyositis/scleroderma Rare
overlap

114. Dermatomyositis: heliotrope rash

115. Dermatomyositis: diffuse facial
erythema

116. Dermatomyositis: rash, chest

117. Dermayomyositis: macular rash and acanthosis
nigricans

118. Dermatomyositis: “mechanic’s
hands”

119. Dermatomyosistis: periungual
involvement

120. Dermatomyositis: nailbed

121. Dermatomyositis and
scleroderma: periungual
involvement (nailfold
capillaroscopy)

122. Dermatomyositis: rash, knees

123. Dermatomyositis: subcutaneous
calcification, knees

124. Steroid myopathy: muscle
(photomicrographs)

125. Inflammatory myopathy
(photomicrograph)

126. Polymyositis: heart
(photomicrograph)

127. Dermatomyositis: calcinosis,
thigh (radiograph)

128. IIM
• Dermatomyositis • Polymyositis
• Peaks in kids and • Any age
older adults • No rash or
• In elderly may be photosensitivity
perineoplastic – • Not perineoplastic
adenoca usually usually
• Rash, photosensitivity • Worse if interstitial
lung disease (anti Jo1)

129. IIM
• Rare
• Diagnosis made by esp proximal muscle
weakness, elevated CK
• Muscle biopsy shows degeneration and
regeneration of m bundles or with
dermatomyositis perivascular inflammation
• EMG – spontaneous fibrillation potentials,
abnormal action potentials

130. Other features
• Heliotrope rash
• Gottren’s sign/papules
• Photosensitivity
• Mechanics hand
• Livedo reticularis
• Some are overlaps with other connective
tissues diseases

131. Antibody and Lab profile
• Increased CK (or aldolase), normal CBC
• Occ increased ESR
• May have + ANA, ENA such as PM/Scl or Jo1
• Jo1 correlates with interstial lung disease and has
a bad prognosis and is very specifici
• PM/Scl- often with scleroderma polymyositis
overlap

135. Treatment
• High doses of steroids
• Steroid sparing drugs such as Imuran,
Methotrexate, Cytoxan
• Treatment and prevention of complications
such as steroid induced osteoporosis
• Biologics possibly (TNF inhibitors)
• Lung disease needs aggressive treatment

136. Complications
• Esophageal involvement – aspiration
• Cardiac involvment – arrhythmia
• Cancer associated – death
• Heterotopic muscular calcification
• Raynauds – ulcers
• Sclerodactyly – flexion contractures