Systemic sclerosis

1. SyStemic
ScleroSiS

2. Case scenario
Manjees,27 yrs
D1/1
Duration of illness-5 years
c/o raynaud’s phenomenon+fingertip ulceration
Not a/w pain,h/o digital gangrene
a/w skin binding down started from B/L forearms
gradually increasing over the course of 4-5 years to
involve whole body
Restriction of hand and wrist movements
Breathlessness
Difficulty in swallowing with vomiting

3. Introduction
• Chronic systemic connective tissue disorder.
• Characterised by thickening of the skin(scleroderma)
and distinctive involvement of multiple internal
organs most notably lungs,GIT, heart, kidneys.

4. Background
• Scleroderma is derived from the greek words
skleros(hard or indurated) and derma(skin).
• Hippocrates first described this condition as thickened
skin.
• First detailed description by Carlo Curzio in 1752.
• Systemic nature of the disease by Robert H.Goetz.

5. Definition
• Multisystemic,autoimmune disease in which there is
increased fibroblast activity which results in
o Vascular damage
o Collagen accumulation
o Scarring of skin and fibrosis

6. Fibroblasts
• Type of cell that synthesises the collagen & extra
cellular matrix.
• Common cells of connective tissue in animals.
Collagen
• Main structural protein in various connective tissues in
animal bodies.
• Found in tendons,ligaments,skin, cornea, cartilage, bone,
blood vessels, gut,inter vertebral disc & dentin.

7. Functions of collagen

8. Systemic Sclerosis
• Increased fibroblast activity resulting in abnormal
growth of connective tissue.
• This causes vascular damage and fibrosis. Fibrosis
occurs in skin, the gastrointestinal tract and other
internal organs.

9. Epidemiology
• Aquired sporadic disease.
• Affects all races.
• Incidence is 9-19 cases/million/year.
• Female predominance(4-5:1).
• Peak age 35 -64 yrs.
• African american > whites.
• Juvenile onset systemic sclerosis is uncommon.

10. Etiology
• Unknown cause.
• Genetic predisposition
• Enviornmental factors
Viruses(cytomegalo virus)
Chemicals(vinyl chloride,pesticides,silca)
Drugs(cocaine,appetite suppressants,vitK)

11. Pathophysiology

13. Classification
According to the extent of skin involvement
• Limited cutaneous systemic sclerosis (lcSSc), or
limited scleroderma
• Diffuse cutaneous systemic sclerosis (dcSSc), or
diffuse scleroderma
• Other types

14. Limited cutaneous systemic
sclerosis
• 70% of SSc cases.
• Milder disease, with less organ involvement
• Slow onset and slow progression
• Affects distal to the elbows and the knee.
• Perioral involvement
• No trunk involvement

15. CREST syndrome
• C- Calcinosis
• R- Raynaud’s Phenomenon
• E- Esophageal Dysmotility
• S- Sclerodactyly
• T- Telangiectasisa

16. Calcinosis in X Ray

17. Raynaud’s Phenomenon

18. Diffuse cutaneous systemic
sclerosis
• 30% of systemic sclerosis cases.
• More rapid onset.
• Skin thickening and Raynaud's phenomenon
occurring together or within a short interval.
• Proximal to elbows and knees
• Involves trunk.
• Internal organ involvement is more common.

19. Limited and Diffuse cutaneous
systemic sclerosis

20. Other types
• Rarer type.
• Includes systemic sclerosis sine scleroderma
• Internal organ involvement without the skin changes

21. Organs involved
• Skin
• Musculoskeletal
• Pulmonary
• Renal
• Gastrointestinal
• Cardiac

22. Clinical manifestations

23. General features
• Fatigue.
• Weight loss.

24. Skin features
Signs in hand
• Raynaud's phenomenon
• Swelling (non-pitting oedema) of fingers and toes
• Hard and thickened skin
• Reduced hand movements- the 'prayer sign(patients
may be unable to place the palmar surfaces together)
• Fingertips may have pitting, ulcers or loss of bulk
from finger pads.

25. • Calcinosis
• Face and mouth:
– Tightening of facial skin.
– Tight lips (microstomia)
• Telangiectasia.
• 'Salt and pepper' appearance of skin, due to areas of
hypopigmentation and hyperpigmentation.
• Dry or itchy skin; reduced hair over affected skin
areas.

26. Musculoskeletal features
• Joint pain and swelling
• Myalgia
• Restriction of joint movement, contractures and
muscle atrophy due to skin sclerosis
• Tendon friction rubs - palpable/audible over the
flexor/extensor tendons of the hands, knees and
ankles.

27. GI features
• Heartburn and reflux oesophagitis.
• Oesophageal scarring and dysphagia.
• Delayed gastric emptying - eg, fullness after meals.
• Esophageal dysmotility - can cause bacterial
overgrowth, malabsorption, diarrhoea and
malnutrition.
• Constipation due to reduced colonic motility.

28. Pulmonary features
• Interstitial lung disease:
– Occurs in as many as 75% of scleroderma patients
– Causes restrictive lung disease
– Causes fibrosis of lungs
– Symptoms and signs: exertional dyspnoea, cough,
coarse basal crackles.

29. Pulmonary features
• Pulmonary arterial hypertension (PAH):
– Occurs in about 10-15% of patients with scleroderma
– A leading cause of death in SSc
– Due to narrowing of vessel as a result of intimal
fibrosis
– Symptoms and signs: exertional dyspnoea,
syncope, right ventricular strain features

30. Renal manifestations
• Scleroderma Renal Crisis
 Abruptly developing severe hypertension-Rise in
SBP by > 30 mmHg, DBP by > 20 mm Hg
 Signs &Symptoms –
oIncrease in serum creatinine by 50% over baseline
or creatinine > 20% of upper limit.
oProteinuria > 2+ by dipstick.
oHematuria > 10 RBC/HPF
oThrombocytopenia < 100
oHemolysis

31. Cardiac Manifestations
• Pericardial Effusion
• Microvascular coronary aretery disease
• Myocarditis
• Arrhythmias and conduction abnormalities

32. Assessment and diagnostic
findings
• Physical examination- clinical manifestations
• Blood tests
• Autoantibodies:
– Antinuclear antibody
– Other autoantibodies in SSc

33. Assessment and diagnostic
findings
• Respiratory system-HRCT,PFT,Chest X-Ray
• GI system-barium swallow,esophageal manometry
• Renal system-urine examination,24 –hr urine protein,
renal function test.
• Cardio vascular system-ECG,ECHO
• Musculo skeletal system-X-Ray of joints,muscle
enzymes.

34. Regular monitoring
• Renal function.
• Lung function tests and chest CT scan.
• ECG and echocardiography.

35. Diagnostic criteria
• The american college of rheumatology criteria for the
classification of systemic sclerosis.
• One major criteria and two minor criteria for
diagnosis.

36. Major criterion
• Proximal scleroderma
 Symmetrical thickening
 Tightening
 Induration of the skin of the fingers and the skin
proximal to the MCP/MTP joints.
 Change may affect the entire extremity,face , neck
and trunk.

37. Minor criterion
• Sclerodactyly-thickening,induration,tightening of the
skin limited only to fingers.
• Digital pitting scars/loss of substance from the finger
pad-due to ischemia
• Bibasilar pulmonary fibrosis-B/L reticular pattern of
linear densities in basilar portions of the lung on
CXR.diffuse mottling/honey comb lung not
attributable to pulmonary disease.

38. Early diagnosis - the VEDOSS
initiative
The VEDOSS (Very Early Diagnosis Of Systemic
Sclerosis) initiative in Europe identified the following
features as being key to diagnosing SSc in the very
early stage:
Puffy fingers in Raynaud's syndrome patients.
Antinuclear antibodies.
Scleroderma-specific antibodies.
SSc pattern on nailfold capillaroscopy.

40. Case scenario
Ba swallow – normal, but pt. was having dysphagia
HRCT – inflammatory lung disease(early)
ANA – diffuse 2+(1:80)
ds DNA – 12.5 IU/ml
2D ECHO – 45 to 50 % EF

41. Physical examinations
• Generalized binding down of skin predominantly the
fingers
• Salt and pepper appearance on nose/ear
• Shortening of digits
• Fixed flexion deformity of interphalangeal joint
• Ulcer in digits

42. Management
• Controlling symptoms
• Preventing complications.

43. Scleroderma Treatment
• Depends on clinical manifestations

44. Monitoring
• Regular review of symptoms.
• Blood pressure monitoring.
• Renal function monitoring.
• Lung function tests and chest CT scan.
• ECG and echocardiography.

45. • To relieve symptoms

46. Raynaud’s
• Calcium Channel Blockers: nifedipine
• Sildenafil (Viagra 0.25 mg TDS) –mainly in patients
with PAH,relaxes blood vessels in lungs
• Parental vasodilators (iloprost) – for severe disease
with impending digital ischemia (25 µg intravenous for
3 to 5 days every 3 months)

47. • Trental (pentoxifylline)
Vaso active drug
Xanthine derivative
Indicated in chronic occlusive peripheral vascular
disorders
Improves blood flow
Starting dose-400 mg BD after meals
Usual dose-400 mg TDS

48. Efficacy and safety of sildenafil in
patients with systemic scleroderma
Aim:To retrospectively analyze the efficacy and safety of
sildenafil (sf) in patients with systemic sclerosis (SS).
Sf was used in 16 patients aged 20-66 years with SS of a duration
of 2 months to 27 years. The indications for Sf treatment were
significant Raynaud's phenomenon (RP) in 3 patients, digital
ulcers (DU) and/or necrosis (N) in 9, pulmonary hypertension
(PH) in 5 and critical ischemia of the left fingers in 1 patient.
RP was seen in all the patients and so the effect of Sf on the
course of RP was evaluated in the whole patient group.

49. RESULTS:There was a significant decrease in the frequency and intensity of
Raynaud's attacks in 11 of the 15 patients treated with Sf.
All 7 patients with DUs showed a decrease in their sizes just within the first
two weeks of treatment.
Complete DU healing was observed within 4-12 weeks of treatment. Pain
ceased just within the first 5-7 days of treatment.
Sf resulted in a rapid reduction in systolic pulmonary artery pressure (sPAP).
In a case, Sf was discontinued because of dizziness after its first intake in a
dose of 12.5 mg. The initial drug intake of the drug was not followed by
adverse reactions in 12 (75%) of the 16 patients. Four patients had Sf-induced
complaints, including headache, dizziness , and more severe angina pectoris .
CONCLUSION:Sf is an effective drug to treat the manifestations of
scleroderma vasculopathy, such as RP, DU/N, and PH. Sf is well tolerated in
most cases. The SS patients with pronounced ECG changes have an increased
risk of severe cardiac events and they need careful ECG monitoring.

50. Gastrointestinal Involvement
Proton pump inhibitor-omeprazole
Mozapride(5 mg TDS)-gastro prokinetic agent,
accelarate gastric emptying

51. Cardiac Involvement
.Pericarditis:
• NSAIDs
• Drainage of effusion if tamponade

52. • Prevent the condition from
progressing

53. • Treatments are used which suppress the body's
immune system
Steroids
Methotrexate
Azathioprine
Mycophenolate mofetil
Ciclosporin
Cyclophosphamide

54. Dexona pulse
• Dexamethasone pulse therapy consists of the
intravenous administration of 100 mg
dexamethasone dissolved in 500 ml of 5%
dextrose on 3 consecutive days. The pulses are
repeated every 4 weeks.

55. • To detect and treat complications early

56. • Skin: dressings and antibiotic medicines are used for
skin ulcers. Surgery for tight skin, nodules or ulcers.
• Lungs: new medicines such as bosentan,
sildenafil, iloprost and have improved the treatment
of pulmonary hypertension.
• High blood pressure and kidney problems are treated
with angiotensin-converting enzyme (ACE) inhibitor.
• Thyroid replacement tablets are given for an
underactive thyroid gland.
• Should receive antibiotics because more susceptible
to infection.

57. • Minimise disability

58. • Exercises to keep joints mobile and muscles strong.
• Various aids such as splints to support the joints, and
help with daily living tasks.
• Regular dental checks if pt.has dry mouth symptoms.
• Patient support groups, such as the Raynaud's and
Scleroderma Association or the Scleroderma Society,
can provide information and support.
• Patient education/self-management programmes.

59. • Nutritional advice, and supplements if needed.
• For Raynaud's phenomenon:
– Prevention - avoid cold and trauma; use warm
clothing or heated clothing(gloves).
– For an attack - warm the body, hands and
feet gently (the skin may be numb and unable to
feel if the heat source is too hot); use gentle arm
movements or gentle massage to help restore
circulation.

60. Case Scenario-Tx history
T. Depin QID 4 monhs
T. Trental 400mg BD 4months
T. Pantocid for 1 year
T.Endoxan 50 BD 4months
T.Betnesol forte –sat&sunday
Mild to no improvement
Increased course of disease leads to increased ulceration

61. Currently –
T. Depin 10 mg BD
T.Pan 40 1 BBF
T.Flexon SOS
T. Endoxan 50mg OD
Condey’s compress
Cold protection

62. Life style and home remedies
• Stay active- Range-of-motion exercises can help
keep your skin and joints flexible.
• Don't smoke- Nicotine causes blood vessels to
contract, making Raynaud's phenomenon worse.
• Manage heartburn- Avoid foods that causes gas.
Avoid late-night meals. Elevate the head of your
bed .Antacids may relieve symptoms.
• Protect yourself from the cold- Wear warm clothes
for protection anytime your hands are exposed to
cold.

63. New initiative
In 4th
systemic sclerosis world congress held in Lisbon
on Feb. 2016 a paper was presented titled Pan PPAR
agonist IVA337 has an anti fibrotic effect in multiple‐ in
vitro and in vivo fibrosis models.
IVA337, is an anti-fibrotic drug with a unique
mechanism of action by activating all three alpha,
gamma, and delta PPARs (peroxisome proliferator‐
activated receptors), receptors that play key roles in
controlling the fibrotic process.

64. Prevention of Digital Ulcers in Systemic
Sclerosis: Real Life Data From the
Observational Study of the EUSTAR group,
Recurrent digital ulcers are a manifestation of vascular disease in
patients with systemic sclerosis. Digital ulcers are usually managed
with endothelin receptor antagonists, such as Bosentan or Sildenafil or
both, iloprost and other drugs, like calcium channel blockers (CCB)
and ACE inhibitors (ACEI).
The study examined the efficacy of Sildenafil,Bosentan,Sildenafil plus
Bosentan,iliprost,and CCB/ACEI in the prevention of new digital
ulcers in systemic sclerosis patients.
268 pts. are assessed, 47 on Bosentan,33 on Sildenafil, 40 on iloprost,
31 on Sildenafil plus Bosentan and 117 on CCB/ACEI alone.
They found that treatment with CCB/ACEI alone was found to be
statistically associated with a 7.1 percent increase in the risk of
developing new digital ulcers, compared to all the other treatments
tested.

65. Effect of Fat and Stromal Vascular Fraction For
Systemic Sclerosis Manifestations in Face and Hands
Systemic sclerosis patients were treated with fat and stromal
vascular fraction — a component of the lipoaspirate obtained
from liposuction of excess adipose tissue that contains
mostly stem cells and growth factors. The growth factors are very
important signaling molecules that can turn on and off
inflammation and cause cells to grow or die.
In total, 14 patients were treated via micro- injection of 16 to 22
cc of fat, and 12 patients’ hands were treated with the stromal
vascular fraction.
They reported that a continuous improvement was observed in
the face of systemic sclerosis patients, primarily a reduction of
pain and improvement in function, and as a consequence, a
noticeable improvement in quality of life.

66. Nursing management
• Altered skin integrity related to inflammation, vaso
constriction
• Altered peripheral tissue perfusion r/t vasospasm and
structural changes
• Joint pain r/t inflammation early in disease
• Joint stiffness r/t inflammation in early diseaseand
contracture in advancing disease

67. Altered skin integrity related to
inflammation, vaso constriction
Assess skin,noting color, moisture, texture, temp, note
redness,swelling or tenderness
Assess skin for any breaks in integrity.Note presence of
any ulcers,size,any drainage and amount of ecrotic
ttissue
Solicit pt’s description of pain expressed as itching or
burning followed by inflammation
Assess interference with life style.Patient may develop
contractures from their tight skin

68. • Provide prophylactic pressure relieving
devices(special mattress,elbow pad)
• Maintain fnl.body alignment
• Clean,dry and moisturize intact skin with warm water
esp. over bony prominences
• Use unscented solution,scented lotions contain
alcohol which dries skin
• Encourage adequate nutrition and hydration,which
keeps the skin moist
• Use non affected skin areas for injection,penetration
may cause further skin damage
• Assist with ADLs as needed
• Tell pt. To avoid direct contact with harsh chemicals

69. Altered peripheral tissue perfusion r/t
vasospasm and structural changes
• Assess hands and feet for color,temp. And skin
integrity
• Remove vaso consticting factors when possible
• Keep extremities warm
• Administer vasodilating medications(Ca channel
blockers)
• Instruct pts. To avoid undue cold exposure
• Wear oven mitts for refrigerator

70. • Wear multiple layers of clothing in cold enviornment
• Wear items made of wool,cotton
• Instruct patient to avoid caffeine and nicotine(cause
vaso constriction)
• Instruct patient in stress management
• Refer to specialized programme as needed

71. Joint pain r/t inflammation early in disease
• Assess for signs of joint inflammation(redness,
warmth, swelling, decreased motion)
• Determine past pain relief measures
• Assess interference with life style
• Administer anti inflammatory medications as
prescribed
• Use non narcotic analgesics as necessary
• Encourage anatomically correct position of joints

72. • Encourage use of ambulation aids when pain related
to weight bearing
• Encourage use of alternate methods of pain control
such as relaxation,guided imagery
• Apply bed cradle to keep pressure of bed covers off
inflamed lower extremities
• Consult occupational therapists for proper splinting
of affected joints

73. Joint stiffness r/t inflammation in early
disease and contracture in advancing disease
• Assess location;generalized or localized
• Assess timing;moning,night,whole day
• Duration of stiffness
• Encourage pt. to take 15 min warm bath on rising
• Encourage to perform ROM exercises after bath
• Allow sufficient time for all activities
• Avoid scheduling tests or treatments when stiffness is
present
• Administer anti inflammatory medications
• Remind patient to avoid prolonged inactivity

74. Thank
you