Approach to systemic

sclerosis
Presenter: Dr. Thalha

Moderator: Dr. Neena Khanna

SR Moderator: Dr. Sindhuja

Introduction
• Systemic sclerosis: multi-system connective tissue disease

• Complex disease: Protean clinical manifestations

• Can overlap with other connective tissue diseases

• High morbidity and mortality

• 2 broad subsets: Limited cutaneous and diffuse cutaneous

Introduction
• Systematic assessment at baseline for organ involvement

• Further triggered testing during follow-up

• Crucial to assess severity to individualise treatment

Multidisciplinary approach
Dermatologist

Rheumatologist Gastrologist

Multidisciplinary
approach

Nephrologist Radiologist

Pulmonologist
 History &
Routine
clinical
investigations
examination

Assess
Long term
Organ
follow-up
involvement
Establishing the diagnosis
• ACR and EULAR criteria have been defined for diagnosis

• Major criteria + 2/3 minor criteria for diagnosis

 Establishing the diagnosis
Item Sub-item (s) Score

Skin thickening of the fingers of both hands extending proximal to the - 9

metacarpophalangeal joints (sufficient criterion)

Skin thickening of the fingers (only count the higher score) Puffy fingers 2
Sclerodactyly of the fingers 4
Fingertip lesions (only count the higher score) Digital tip ulcers 2
Fingertip pitting scars 3
Telangiectasia - 2
Abnormal nail fold capillaries - 2
PAH/ILD (maximum score is 2) PAH 2
ILD 2
Raynaud’s phenomenon - 3
SSc related autoantibodies (maximum score is 3) Anticentromere 3
Anti-topoisomerase I
Anti-RNA polymerase III
• Long history of Raynaud’s phenomenon
• Anticentromere antibodies
• Rare & late visceral involvement
• More vascular problems
• Absence of tendon friction rubs
• ILD less common
• PAH more common
• Short history of Raynaud’s phenomenon
• Antitopoisomerase-1 antibodies
• Early & frequent visceral involvement
• More problems due to fibrosis
• Presence of tendon friction rubs
• ILD more common
• PAH less common
Establishing the diagnosis
Establishing the diagnosis
Clinical features of MCTD

Raynauds phenomenon Arthritis and arthralgia

Puffy fingers/hands Myositis

Sclerodactyly Serositis

Oesophageal involvement Anaemia/lymphopenia

PAH/ILD High titres of U1RNP Ab

General symptoms
Early phase: clue to think about CTD

General symptoms in systemic sclerosis

At any point of time
Fatigue 65-76%
Fever 50%
Weight loss 45-50%

Must be interpreted with caution

• Higher Heart rate & Low resting BP: autonomic dysfunction, vasodilators

• Postural hypotension

• Malnutrition: disease, socio-economic morbidity, drug & immune suppression

related loss of appetite
 Evaluation
Test Finding Interpretation
CBC Anemia: Normocytic > microcytic > macrocytic Anemia of chronic disease > IDA >
Malabsorption

Microangiopathic hemolytic anemia + Thrombocytopenia Scleroderma renal crisis

RFT Raised creatinine Renal involvement, rule out
scleroderma renal crisis
Renal crisis: increase in creatinine >50% over baseline or
>120% of UNL
LFT Decreased total protein Malnutrition
ANA Positive 95% cases have ANA +
ESR/CRP Raised During active phase of disease
Test Finding Interpretation

Creatine Elevated Myopathy/Myositis

Kinase

Urinalysis Proteinuria/ cellular cast Renal dysfunction

Iron, vitamin Low levels Malabsorption/Malnutritio
A, B12 and n
folic acid

Auto-Ab Anti-Scl 70 dcSSc > LcSSc, increased

ILD

Anti-centromere lcSSc >> dcSSc

Anti- RNA P III dcSSc >> LcSSc, renal
involvment
Cutaneous involvement
Early phase:

∙ Diffuse erythema & non-pitting oedema of skin

∙ Binding down of skin
∙ Painful puffy fingers
∙ Telangiectasia: Mat-like
∙ Pruritus: 40% of patients & may be intense during the early or active stages
of disease
Cutaneous involvement
With disease progression:
∙ Tapering of fingers
∙ Reduced hair growth, sweating
∙ Xerosis
∙ Fingertip – ulcers/ pitted scars/ gangrene
∙ Ulceration over DIP & PIP due to repeated microtrauma

• Rapid progression: particularly in diffuse disease

• Extension proximally to the MCP joints: Important

Digital ulcer: True ulcer vs traumatic ulcer

Cutaneous involvement
Facial changes:
• Mat-like telangiectasia
• Beak-shaped nose
• Reduced aperture of the mouth
• Radial furrowing around the mouth
• Expressionless, mask-like stiffness

Rarely patinets have no skin induration: SSc sine scleroderma

Pigmentary changes

Hyperpigmentation

Salt-and-pepper pigmentation

Scar depigmentation
Calcinosis cutis
• Calcium deposits in skin & soft tissues

• Firm papules/nodule
• Ulcerate
• Extrude chalky material

• Sites:
• Volar aspect of fingertips
• Palms
• Extensors of forearms
• Olecranon & prepatellar bursae
• MCP & IP joints 🡪 ulcerate 🡪 chalky material
Evaluation for cutaneous involvement
For skin thickening:
• mRSS: Modified rodnan skin score
• Ultrasonography of the skin
• Rare: Skin biopsy

For Calcinosis cutis

• Ultrasonography and plain X-ray 🡪 first line investigations
• Other investigations: MRI, CT & Diffraction enhanced X-ray
Modified rodnan skin score
• 17 sites

• Max score/site: 3

• Max total score: 51

• Score > 14 likely s/o dcSSC

Modified rodnan skin score
Score 0: Normal thickness

Score 1: Thickened skin, movable

Score 2: Able to pinch, but not

movable

Score 3: Inability to pinch

Ultrasonography of the skin
• Non-invasive assessment of dermal thickness using High frequency probe (18-50 MHz)

• High reproducibility: intra and inter-observer variability 4 & 8 % respectively

• High sensitivity: detect early & measure change

• Done at 17 sites similar to mRSS and corelates with mRSS

• In research settings/clinical trials

Dermo-epidermal thickness is
measured
For calcinosis cutis
When in doubt/ sub-clinical: Ultrasonography and radiographs

USG: linear hyperechoic lesions with distal acoustic shadow

Plain-Xray: Calcific densities

Other investigation: MRI, CT and diffraction enhanced X-ray

imaging
Raynaud's phenomenon
• Triphasic > biphasic

• 90-95% patients of SSc

• Other site: ear, nose

• Rule out other causes

• Primary vs secondary

• Look for other changes

Raynaud's phenomenon
Predictors of Raynaud’s phenomenon being secondary:
• Abnormal nail fold capillaries
• Asymmetric intense attacks
• Late age of onset (30 years)
• Ischemic skin lesions +
• Presence of ANA/ anti-topoisomerase, anti-centromere antibodies
Nail Fold Capillaroscopy
3 stages
• Early: Dilatation
• Active: Giant capillaries &
microhemorrhage
• Late: loss of capillaries &
neovascularization

Correlates with severity and

progress of pulmonary
involvement
Raynaud's phenomenon
Scleroderma-Dermatomyositis pattern (Modified Maricq’s criteria)
2 or more of the following in at least 2 nail folds

a) Enlargement of capillary loops

b) Loss of capillaries Sensitivity ?
c) Disorganization of the normal distribution of capillaries Specificity ?
d) Budding (bushy) capillaries
e) Twisted enlarged capillaries
f) Capillary hemorrhages (extravasates)
Raynaud's phenomenon (RP)
attack diary

14 pages

Patients record
• The duration (in minutes)
• The number of times they
were exposed to cold
temperature
• Raynaud's Condition Score
(RCS) each day for 14 days
Thermal Imaging • Indirect method to detect RP

• Using infrared thermal imaging

• In RP: fingertips/toes are cooler

than centre of palm/sole

• 2.2 C and 3.1 C for hand and

feet respectively

• Cold & Hot test can also be

done
Thermal Imaging
10 minutes post immersion in cold water 15C for 1 minute

Control Case of SSc

• In control, rewarming of fingetips occurred and it reached 33C

• In SSc, fingertips were 21 C and also cooler than centre of palm
Gastrointestinal involvement
• Symptoms because of abnormal motility or malabsorption

• Neurological abnormality 🡪 muscle dysfunction 🡪 atrophy 🡪 fibrosis

Oral cavity:
• Microstomia and microcheilia (50-80%)
• Xerostomia (30-40%)
• Mandibular resoption
 Symptoms
Dysphagia
Oesophageal involvement Odynophagia
Heartburn
Regurgitation
Chronic cough
Most affected organ Hoarseness of voice
• Due to reduced sphincter pressure, hiatal hernia, dysmotility

• Seen in dcSSc > lcSSc

• Dysphagia: reduced peristalsis, GERD, candidiasis

• Esophagitis seen in 60% of SSc

Oesophageal involvement
Gastric involvement
Gastroparesis: early satiety, bloating, nausea, vomiting
Barium meal: dilated and atonic stomach
No visible obstruction

Gastric antral vascular ectasia: Iron deficiency and acute GI bleed

Upper GI endoscopy: Diagnosis and management
Small bowel involvement
Symptoms and signs of
Chronic intestinal pseudo-obstruction (CIPO) acute or chronic bowel
Impaired gastrointestinal propulsion obstruction in the
absence of any
Barium: flocculation, pooling mechanical obstruction
Manometry: uncoordinated & minimal MMC

Nausea
Vomiting
Small intestinal bacterial overgrowth (SIBO): Diarrhoea
Impaired motility 🡪 stasis 🡪 bacterial overgrowth Abdominal distension
Bloating
43-56% of SSc patients Malabsorption
Methane breath test Vitamin B12 deficiency
Normal folate levels
Culture: Stool/Jejunal aspirate
Small bowel involvement
Pneumatosis cystoides intestinalis
• Presence of air in the submucosa or sub-serosa ( bacterial overgrowth)
• Can rupture to form pneumoperitoneum
• Xray/CT show radiolucent cyst

Jejunal diverticula
• May cause intestinal obstruction if mouth is large
• Barium meal follow though and CT-enterrography
Colon & anorectal
Colon involvement: 20-50% patients
Delayed transit time 🡪 constipation 🡪 bacterial growth 🡪 malabsorptive diarrhea

Anorectum: Internal anal sphincter affected

Vascular insufficiency: faecal incontinence, rectal prolapse

Any new onset constipation: Do a DRE, colonoscopy evaluation, MRI and anorectal
manometry as needed
Proposed pathway for screening and managing malnutrition

Malnutrition universal screening tool

MUST: Malnutrition universal screening tool
Symptoms Investigations to be done
Dysphagia, odynophagia, heartburn, Barium swallow – 1st line
regurgitation, chronic cough, Others: Manometry, 24ph monitoring,
hoarseness of voice endoscopy

Early satiety, bloating Barium meal

Acute iron deficiency anaemia Upper GI endoscopy
Abdominal distention with bloating & Xray of the abdomen, Barium meal
Constipation follow-through, small bowel manometry

Abdominal distention with bloating & Stool culture, stool acidity testing,
diarrhoea with signs of malnutrition Jejunal aspirate culture

Constipation DRE, Colonoscopy, Anorectal

manometry
Pulmonary involvement
• >70% patients of SSc

• Main manifestations: ILD & PAH

• Other manifestations
• Pleural effusion
• Pneumonitis: aspiration or drug induced
• Spontaneous pneumothorax
• Lung malignancies
Interstitial lung disease
• Present with fatigue, exertional dyspnoea, dry cough

• Rarely: chest pain and haemoptysis

• In the EUSTAR trial, 35% of deaths were directly attributable to ILD

Risk factors for severe ILD

Diffuse cutaneous systemic sclerosis (70% in dcSSc vs 40% in LSSc)
< 3 years disease duration
Severe gastro-oesophageal disease
Presence of anti-topoisomerase-1, anti-th/to rnp, anti-U11/U12 antibody
Rapid decline of lung function

Investigations in ILD
Investigation Use in ILD
PFT To determine Lung capacity ,
airflow obstruction , gas
exchange
HRCT Detailed cross-sectional imaging
of lung parenchyma
6MWT Exercise tolerance
LUS USG characterisation of lung
Modified medical research council scale: to grade dyspnoea
Is Chest x-ray
necessary if a HRCT
is being done?
Salient feature
Useful in monitoring progress
and response to treatment.
High sensitivity , specificity and
accuracy to diagnose ILD
Non-invasive, simple,
independent predictor of
mortality
Non-invasive , non-ionising ,
bedside procedure to detect
fibrosis of lung
Modified medical research council
Pulmonary function test
Restrictive pattern: Low FEV1 & FVC

PFT Significance
FVC <80% (earliest)
FEV1/ FVC ratio Normal or increased
DLCO Decreased Most significant marker of poor outcome
FVC/DLCO > 1.4-1.6 PAH

Indicative of disease progression: a decline from baseline of

• 5–10% in FVC
• 10–15% in DLCO
Pulmonary function test
• Rate of false-negative results is high: suited for monitoring than early detection of
the disease

• Used to assess the severity and monitor the course of ILD

• Difficult to interpret due to the wide normal range (80 to 120% of predicted)
High resolution computed tomography
• Established & reliable imaging modality to detect & characterize ILD

• Schurawitzki et al., detection ability of HRCT is better than radiographs (91% vs

31%) for ILD

• ILD is present on HRCT in 55% of patients on initial evaluation but the prevalence
is higher (96%) among patients with abnormal PFT results
High resolution computed tomography
Pattern of ILD
NSIP – most common pattern in SSc-ILD (77.5%)
Characterised by homogenous inflammation with varying fibrosis
Two variants - Fibrotic(76%) and cellular (24%)

UIP – second most common pattern (7.5%)

Distortion of lung architecture and presence of fibroblastic foci

Other less common patterns are Organising pneumonia (OP) and diffuse alveolar
damage (DAD)
Ground glass opacification, reticular opacity and micro-cysts
Lung ultrasound
• Useful to explore the pleura and peripheral lung regions

• Interstitial abnormalities: represented by B-lines

• B-lines consist of discrete laser-like vertical hyperechoic reverberation artifacts

• <5 B-lines rule out ILD

• An accessible, low-cost, non-ionizing, reproducible tool that is highly sensitive for

ILD screening
Normal lung Pathological lung

★: Pleural irregularity

B-line
B-Lines
 Strong clinical pointers:
Pulmonary artery hypertension Syncope
Light-headedness
Orthostasis
• Presents as: dyspnoea at rest, syncope, palpitations, fatigue Angina/chest pain

• 10-12% patients, accounts for 35% mortality

• Mean Pulmonary artery pressure ≥ 25mm Hg + Pulmonary capillary wedge

pressure of ≤ 15mm Hg in right heart catheterization

• Can be primary or secondary due to ILD

Investigations for PAH Loud P2
RV lift over precordium
Advanced: TR murmur, ↑ JVP
• Physical examination

• ECG: Right-axis deviation, right atrial & ventricular enlargement

• Chest X-ray: Loss of peripheral vascular markings, filling of the retrosternal space
(RV enlarged)

• Specific, but insensitive

Investigations for PAH
DLCO: Isolated reduction
• Reflects integrity of pulmonary vascular bed
• Low and decreasing values: predictive of PAH and poor outcome

Doppler echocardiography: routine baseline screening

• TR jet >2.5 and Right atrial/ventricular enlargement

Right heart catheterisation: Gold standard

6 minute walk test
• To test exercise tolerance

• Baseline: HR, BP, Spo2 and Borg scale

• Can rest/lean on wall in between

• Post-test: Calculate distance, Repeat HR, BP, Spo2 and Borg scale

• Uses: Monitoring, treatment goals

Renal involvement
• Dysfunction due to vasculopathy: usually benign

Isolated proteinuria
• Mostly subclinical Isolated GFR reduction
Scleroderma renal crisis
ANCA-vasculitis
• 5 to 15% of patients

• dcSSc > lcSSc

Renal involvement
• Proteinuria: marker of renal vascular disease Proteinuria > 1g: uncommon
Present before reduction of GFR Think of underlying glomerular
disease
ACEi lead to significant reduction

• Isolated GFR reduction: glomerular hypo-filtration

Present before elevation of serum creatinine
eGFR <60ml/min/1.73 m2 🡪 3-fold increase in mortality
 Headaches
Seizures
Scleroderma renal crisis Fever
Malaise

• Most specific, 5-10%, dcSSc > lcSSc

Hypertension
Acute >140/90mmhg
• Life threatening, high mortality rates Encephalopathy
Retinopathy

• Acute ↑ in creatinine (>50% over baseline)

• New onset microangiopathic haemolytic anaemia, thrombocytopenia
• Use of prednisolone >15mg/day in last 6 months

Screening: 3 monthly
Serum creatinine
• Rx: ACEi, Captopril & Enalapril Urine protein
Blood pressure
Tendon friction rubs
ANCA-vasculitis
Renal failure
Milder HTN
• Subacute presentation Proteinuria

• lcSSc > dcSSc

• In late stage of SSc

• Rx: Oral steroids (doesn’t respond to ACEi)

Cardiovascular involvement
Pericardial
Myocardial
• Secondary > primary Arrythmias

• 10-20% symptomatic 🡪 poor prognosis

• Severity: M > F

• 5-year mortality rates: 75%

Pericardial involvement
• Usually clinically silent and benign

• Can present as
• Pericarditis (most common)
• Pericardial effusion
• Tamponade

• Large pericardial effusion: ↓ cardiac output 🡪 renal hypoperfusion 🡪 renal crisis

Myocardial involvement
• Myocardial fibrosis: Patchy (hallmark)
LV dysfunction diastolic > systolic
Myocardial fibrosis
Myocardial Ischemia
• Myocardial ischemia: increased risk
Due to frequent vasospasm of small vessels
 Myocardial fibrosis:
Conduction abnormalities Tachyarrythmias

• Due to fibrosis of myocardium and conduction system

• 50% - resting ECG normal, but arrythmia on exercise

• Left Anterior Fascicular Blocks > AV-blocks Conduction system fibrosis:

Bradyarrythmia
Conduction defects
• Sudden deaths: ventricular arrythmia
Evaluation of cardiac involvement
Screening modalities
• ECG
Signs of cardiac involvement
• ECHO BNP > 60pg/ml
NT-proBNP > 125pg/ml
• Natriuretic peptide: BNP and NT-proBNP

Early detection and to assess response to therapy

• Cardiac MRI
ECG
Holter monitoring: Palpitations, light
headedness, dizziness, syncope

Treadmill ECG: if exertional palpitations

Left Anterior Fascicular Blocks > AV-blocks

Cardiac MRI
∙ Gold standard for assessment of cardiac volumes

∙ Provides the most accurate and reproducible quantitation of ventricular mass

and ejection fraction

∙ Ideal for asymptomatic patients, follow-up, and response to therapy

∙ Assessment of pulmonary hypertension and its complications

∙ Does not use ionizing radiation

 Muscle weakness
Arthritis
Musculoskeletal involvement Tendintis
Tendon friction rubs
Joint contractures

• Early: arthralgia, myalgia & fatigue

• Muscle weakness: 90% cases

a) Low grade myopathy
b) Inflammatory myositis (overlap r/o MCTD/DM)

• Joint pains > Arthritis (polyarticular)

Leads to immobility & contractures (mostly small joints)

Tendon friction rub: feeling of coarse crackling or crepitus

Associated with aggressive disease & renal crisis
Evaluation for muscle involvement
If suspecting proximal muscle weakness
Measure levels of CPK, LDH & aldolase
EMG
Normal enzyme levels don’t rule out myopathy MRI
Muscle biopsy

EMG: muscle membrane irritability

Action potentials: small short & polyphasic

MRI: Oedema, fibrosis & calcification

New techniques: Contrast enhanced USG, DWI MRI

No definite criteria
Evaluation for skeletal involvement
X-ray
USG
• Flexion contracture: 90% MRI
Doppler USG
Finger involvement: dcSSc > lcSSc ESR/CRP

• Joint space narrowing: due to synovitis or osteoarthritis

Most frequently in DIP Juxta-articular
osteopenia and
osteoporosis
• Acro-osteolysis: distal phalanges (9-63%)

• Erosion: RA-like can be seen, if predominant 🡪 do RF & anti-CCP

Flexion contracture Acro-osteolysis of thumb and calcinosis
Sexual dysfunction
• Male erectile dysfunction: 81%
PDE5i: Tadalafil preffered

• Female: Lower levels of sexual functioning

Tight skin around introitus
Joint contracture
Muscle weakness
Investigations
• Baseline routine investigations:
• Hemogram
• Blood chemistry: LFTs, RFTs
• ESR, CRP, ANA & Autoantibody profile
• Urine: routine microscopy, 24-hour protein
• Chest X-ray
• ECG
Investigations
Baseline assessment of commonly affected systems for every patient of SSc

• Cutaneous: mRSS scoring, Nail fold capillaroscopy

• Gastric: Barium swallow

• Pulmonary: Pulmonary function tests with DLCO, 6-minute walk test, HRCT, 2-D ECHO
to look for pulmonary parameters

• Cardiac: 2D-Echocardiogram and if warranted 24-hour holter monitoring

• Renal: GFR, Renal doppler

Follow-up investigations
Organ system Diagnostic technique Repeated
Skin Modified Rodnan score 6 monthly
Ultrasonography of skin
Renal GFR, Creatinine clearance, U. protein 6 monthly
Renal doppler 12 monthly
Gastrointestinal Barium swallow 12 monthly
Pulmonary PFT/DLco 6 monthly
ILD HRCT 12 monthly
PAH 2D echo, 6 min walk test 6 monthly
Cardiac Standard ECG, 24-hour Holter, 2D echo 6 monthly
Raynaud’s phenomenon Nail fold capillaroscopy 6 monthly
Management
• As dermatologists we mainly treat the cutaneous and vascular symptoms and to
a limited extent other systems

• Management: Targeting disease and symptoms

• Most drugs target inflammation and fibrosis underlying the pathogenesis:

beneficial for >1 organ system
General measures
• Cold protection
• Gloves, socks
• Luke-warm water for chores

• Stop smoking

• Manual lymphatic drainage & exercise

Management of cutaneous involvement
• Skin binding down Diffuse skin involvement: severe or progressive

1st line: Tablet Methotrexate 0.2-0.3mg/kg/week (Level of evidence Ia)

2nd line: Tablet MMF 2-3g/day

Other options:
Dexamethasone pulse
Dexamethasone-cyclophosphamide pulse
Cyclophosphamide pulse
Azathioprine
PUVA/UVA1
Management of cutaneous involvement
• Skin dyspigmentation Cosmetic reasons
Topical hydroquinone cream
Topical steroids
Topical retinoids
Camouflage
Salicylic acid and chemical peels

• Telangiectasia
Flash lamp pumped pulsed dye laser
Intense pulse light
Microstomia Pre-fat transplant

Post-fat transplant
Both groups: 0.6-0.8 cm increase in mouth opening
Calcinosis cutis
• Diltiazem: potential preventive effect (180-480 mg/day)

• Rituximab: partial response

• Minocycline 50-200mg/day: partial response

• Intralesional sodium thiosulphate: partial response

• Co2 laser and ESWL: last line

Before After
A case of CREST syndrome with refractory calcinosis cutis
Intralesional Sodium thiosulphate 12.5g/50ml 0.1-0.2 ml/lesion three weekly x 3
No improvement noted
Raynaud's phenomenon
Retrospective study with 3 year follow-up

15 patients with severe RP

Botox A 20 IU/ml @ base of lateral aspect of fingers

Follow-up ( 30min, 7 day, 3m, 6m & annually) Usually not given in thumb: least affected
Most susceptible for muscle weakness
5/7 basal ulcers healed at 3 months Good improvement:
No. of patients Time
No serious adverse effects 4/15 30 min
8/15 1 month
9/15 1 year
 Microvascular surgery reserved for:
Refractory RP
Refractory digital pain
Non-healing digital ulcer

Digital sympathectomy: stripping of adventitia: mainstay

If segmental occlusion of ulnar/radial artery: Bypass/arteriolysis

Improves digital perfusion, heal ulcers & eliminate pain

Healing occurs within 4-6 weeks

 Critical digital ischemia/ digital ulceration
Management Evidence
Hydrocolloid occlusion, wound care, pain control and antibiotics
Parenteral Prostacyclin: iloprost (healing) Ia
ERB: Bosentan (reduction in new ulcers) Ia

PDE5 inhibitor: Sildenafil 25-50mg TDS (healing) IIa

Combination of ERB + PDE5 inhibitor + ARB III
Statin III
Antithrombotic therapy III
Surgical approaches:
Radial micro-arteriolysis III
Botulinum toxin injection III
Sympathectomy III
 General measures:
Have meal 2 hours before sleep
Management of GI involvement Sleep with head end propped up

• Based on symptoms: EULAR 2017 recommendation

• Severity of symptoms ≠ functional impairment

• PPI: for reflux, to prevent ulcer & stricture Tablet. Pantoprazole 40mg OD BBF
• Long term use can cause nutritional deficiency

• Prokinetic drugs: for symptomatic motility disturbances (dysphagia, early satiety,

bloating, pseudo-obstruction)
• All available prokinetic drugs can be applied based on individual consideration

Tablet. Mozapride 5mg BD-TDS

Management of GI involvement
• Intermittent or rotating course of broad spectrum antibiotics with activity against
both aerobic and anaerobic enterobacteria for SIBO Quinolones
Amoxicillin-clavulanic acid
Metronidazole
Neomycin or
Doxycycline

• Other conditions might need a gastroenterologist referral

 Indication to treat
• HRCT: >20% lung

Management of ILD involvement

• FVC < 70%
• Significant decrease in last
Mild disease 12 months
• FVC > 10% or decrease
• MMF: 2-3 gm per day for 3-5 years in DLCO > 15%

Moderate to severe disease

• Monthly cyclophosphamide pulse: 500- 750 mg/m2 for 6-12 months
• Followed by MMF or Azathioprine for 3-5 years
• Rituximab: 1-2 cycles followed by maintenance therapy with other agents
• Steroids as dexamethasone pulse: not much evidence for ILD
Management of PAH
Multidisciplinary approach
1st line: Endothelin receptor antagonists
Tablet Bosentan 62.5mg BD
Tablet Ambrisentan 5-10mg OD
2nd line: PDE 5 inhibitors
Tablet Tadalafil 40mg/kg
Tablet Sildenafil 20mg TDS
3rd line: Soluble Guanyl cyclase stimulator
Tablet Riociguat 1mg TDS
Severe cases: Prostacyclin analogue
Epoprostenol 2ng/kg/min IV
General measures:
Daily exercise
Routine vaccination
Avoid tobacco/marijuana
Avoid pregnancy
Indications to treat:
• Pulmonary arterial pressure >25 mm Hg
• Pulmonary capillary wedge pressure <15 mm Hg
• Diameter of pulmonary artery > 28 mm
• Isolated reduction of DLCO to <65%
• Ratio of FVC/DLCO >1.6
Management of renal involvement
• Proteinuria and Reduced GFR: ACEi Tablet. Captopril 12.5-25mg BD

• ANCA-Vasculitis
IV cyclophosphamide + corticosteroids
Maintenance with MMF/Azathioprine/Mtx

• Scleroderma renal crisis

Manage BP: ACEi ± CCBs Target <130/90 mmhg
Dialysis/Renal replacement therapy
 Management of cardiac involvement

Manifestation Treatment
Coronary artery disease CCB or ACEi/ARB with statin, coronary stenting, anti-platelet therapy
LV diastolic dysfunction Treat Heart failure symptoms
RV dysfunction Digoxin, diuretic for heart failure
LV systolic dysfunction Beta-blocker or CCB or ACEi/ARB
Pericardial effusion No treatment indicated unless symptomatic; rule out renal crisis;
Management of cardiac involvement

Manifestation Treatment
Constrictive pericarditis Digoxin, diuretic for heart failure. Pericardial stripping is C/I
Myocarditis Cyclophosphamide, intravenous pulse steroids
Tachyarrhythmia Centrally acting calcium channel blockers (diltiazem,
verapamil); Beta-blockers avoided if Raynaud’s is present;
can consider ablation or defibrillator
Bradyarrhythmia Pacemaker