APPROACH TO

SCLERODERMA

D R . A S H I S H J A G AT I
A SS O C I AT E P R O F E SS O R ,
S C L H O S P I TA L & N H L M E D I C A L C O L L E G E ,
AHMEDABAD
 Our approach

Differentiation b/w various CTD on basis of

clinical feature

Investigations in CTD
 General
 Specific
 Systemic complications
 Approach

Connective
tissue
disease

Sjogren’
LE s
Myositis Others
group syndrom
e
Scleroderm
a Overla
p

Systemi
DLE ScLE SLE Morph MCTD
c
ea
sclerosis
 Clinical features

Rash + Dryness of
Rash – eyes,
Binding mouth Multiple
down – Binding features
down of salivary
Photosensitiv skin + gland
ity + swelling
Overla
LE/ p
Dermatomyositis Sjogren’s
syndrome

Malar Heliotro
Scarrin pe rash
g with rash Raynaud’s
Raynaud’s
atrophy Arthralgia Gottron’s –
+
Non papules Patchy
Adhere Oral Generalised
nt scarring ulcers Mechani involvemn
tightening
scaling annular cs hands t of skin
Systemic of skin
polycyclic Proximal with lilac
Cicatric or involveme Oesophagea borders
ial nt muscle l dysmotility
paulosquam weaknes
alopeci ous rash Facial
a s
predominan changes
tly on upper Morphea
trunk
Systemic
sclerosis
SLE
Dermato
DLE ScLE myositis
Limite Diffus
d e
 History

Swelling/history of swelling of the hand, feet

(Acute/chronic) and digital ulcerations
Skin tightness ( Onset and Progressive)
History for Raynaud phenomenon
Difficulty in opening of mouth, dysphagia or dyspepsia
Joint pain, swelling or morning stiffness
( more with diffuse disease)
Muscle pain, weakness ( more with diffuse disease)
Anorexia, fatigue or weight loss
Dyspnoea on exsertion or rest
Relevant question to rule out other condition that may
have similar features (pseudoscleroderma)
 Raynaud’s phenomenon

Episodic symmetric, acral vasospasm

characterized by pallor, cynosis and erythema
and a sense of fullness or toutness, which may
be painful.
Pallor (Vasospasm) cyanosis (prolonged
lack of oxygen) erythema ( on rewarming
blood vessels reopen causing local flushing)
Should be differentiated with primary
Raynaud’s.
Should know Cold provocation test.
Facial changes
Mask like facies
Conjunctival sign
Pinched up and
beaking of nose
Telangiectases
Restricted mouth
opening
Radial furrowing
around mouth
Prominent chin,
zygomatic bones
Salt and pepper
pigmentation
 Loss of finger pads,
Terminal digit resorption
Reduced movement
Fixed deformity

Digital pitting scars

 Skin Induration
Should know
Modified Rodnan skin scor
20 MHz ultrasound

Acrosclerosis
Calcinosis cutis
Nail changes
Normal capillary “loops” (U- shaped
structures compared to hairpin)

Capillary drop out

• Diffuse systemic sclerosis

Capillary dilatation
• Limited systemic sclerosis

Thickened,ragged cuticle with capillary

dilatation
• Dermatomyositis
• SLE
Joint manifestations
Scleroderma
 Acro osteolysis
 Erosive arthropathy with
‘pestle and mortar’
deformity of DIP
 Increased intraosseous
deposition of calcium
 Osteopoilkilosis
 Multiple dense islands of
bone occur at epiphyses
and metaphyses
 Calcinosis
 Systemic examination

Respiratory

CVS

CNS

GI
 Diagnosis Criteria
[American college of rheumatology(ACR)/European league
against rheumatism(EULAR)]
Item Sub-item Weight/
score
Skin thickening of the finger of both hands - 9
extending proximal to the MCP joints
(sufficient criteria)
Puffy fingers –or- 2
Skin thickening of finger sclerodactyly 4
Digital tip ulcer – or – 2
Fingertip lesion Pitting scar 3
Telengietasias - 2
Abnormal nail fold capillaries - 2
Pulmonary arterial hypertension-or- 2
Interstitial lung disease 2
Raynaud phenomenon - 3
SSc-related autoantibodies Anti-centromere,anti- 3
topoisomerase I,Anti-RNA-
polymerase III
Investigations
 Investigations

Baseline/ Specific for Systemic

General CTD complications

ECG,CXR,Ba
ANA HPE DIF studies, kidney
function
 Baseline/General investigations

 CBC- Anemia
 SLE-iron deficiency,hemolysis,renal failure
 SSc- Malabsorption, GI bleed, Renal failure
 Leucopenia
 Thrombocytopenia
 Raised ESR
 False positive syphilis serology-SLE>DLE>SSc
 Positive Coomb’s test – can occur in absence of hemolytic
anemia SLE>>DLE>SSc
 Proteinuria SLE>SSc
 Rheumatoid factor SLE>SSc>DLE
 Investigation
s

Specific for Systemic

General
CTD complications

ECG,CXr,Ba
swallow,
ANA HPE DIF
kidney
function
 Antinuclear antibodies

•A NT I - D N A A N T I B O D I E S
•A NT I - E N A A N T I B O DI E S
• Anti-Sm; Anti-Ro; Anti-La; Anti-RNP; Anti Scl70,;Anticentromere
•A NT I - P H O S P H O L I P I D A NT I B O D I E S
• Anti-cardiolipin antibodies; lupus anticoagulant; anti-β2 glycoprotein 1 antibodies
 ANA patterns

homogeneous peripheral

speckled nucleolar
 ANA

Rim Homogenous Speckled Nucleolar

dsDNA, Fine Discrete

anti
histone
Anti U3
dsDN Sm Ro,La U1RNP Scl RNP,anti RNA
70 Centro
A Polymerase 1
mere
SLE SLE,
Drug SLE SSc, SLE dSSc
SLE
induced Sjogr MCTD LSSc Scleroder
LE en’s ma
 Anti-ENA

A group of different auto antibodies

Probably no correlation with disease activity

ANA Vs Anti ENA
ANA tests for the presence/absence of
autoantibodies while the ENA panel evaluate
which proteins in the cell nucleus the
autoantibodies recognise
 Anti-ENA

 Anti-centromere antibodies
 Associated with limited sclerosis
 Anti-topoisomerase I antibodies (anti-Scl-70)
 Associated with progressive systemic sclerosis
 Anti-Jo-1 antibodies
 Associated with poly-or dermatomyositis
 Anti Mi-2 (highly specific)
 Anti SRP
 Anti PL12
 Anti Ku
Anti-Pm-Scl – polymyositis-scleroderma overlap

Anti-fibrillarin (anti –U3 ribonucleoprotein)-

diffuse cutaneous ss, Pulmonary hypertension and
renal disease

Anti Th/To- limited skin involvement, renal crisis,

pulmonary hypertension

Anti-RNA polymerase III – Rapidly progressive

skin involvement, renal crisis.
Histopathology
 Histopathology

Histologic evaluation
essential for proper
Immunofluorescence evaluation of connective
tissue disease
Serologic evaluation
 Scleroderma

 It is important that the specimen biopsy include adequate

amount of subcutaneous tissue, since most of the diagnostic
alterations are observed there

 Early inflammatory stage

 Intermediate phase
 Late sclerotic stage
 Scleroderma

 Early inflammatory stage-

 Moderately severe inflammatory cell
infiltrate, predominately
lymphoplasmacytic
 Between collagen bundles and
around blood vessels
 Infiltrate may extend into
subcutaneous fat and project around
eccrine glands
 Wavy collagen fibres within the
reticular dermis and s/c fat
 Vascular changes in form of
endothelial sweeling
 Scleroderma

 Late sclerotic stage

 Inflammatory infiltrate has disappeared

 Collagen is closely packed and eccrine glands appear atrophic or

absent

 Collagen may replace fat cells in the subcutaneous tissue

 Few blood vessels are noted here, they often have fibrotic walls with
narrowed lumen
Morphea
 Investigation
s

Specific for Systemic

General
CTD complications

ECG,CXR,Ba
swallow,
ANA HPE DIF
kidney
function
 ECG & Chest X Ray

•ECG
• SLE – features s/o pericarditis
• SSc - atrial fibrillation/ atrial
flutter
 SLE Systemic sclerosis

• Pleural thickening Fibrotic changes involving the

• Infiltrations lower two thirds of the lung,
• Shrinking lung syndrome with associated volume loss
and honeycombing
 Barium studies
SLE Systemic sclerosis

Impaired contraction in upper part Dilated atonic air containing lower

of oesophagus Oesophagus
Should know
Oesophageal manometry &
Szintigraphy
Watermelon stomach/GAVE

Barium enema
shows wide mouthed
diverticula
 Renal involvement in SLE v/s SSc

SLE SSc

•Wire loop lesions •Triad of

• Thickening and hyalinisation of • Intimal proliferation of small
capillary basement membrane intralobular arterioles
• Localised to one part of • Fibrinoid necrosis of walls of
glomerulus afferent arterioles
• Cortical infarctions

Class I, minimal mesangial LN;

Class II, mesangial proliferative LN;
Class III, focal LN;
Class IV, diffuse segmental LN;
Class V, membranous LN; and
Class VI, advanced sclerosing LN.
 Management of cutaneous features
 Cutaneous sclerosis- UV phototherapy, minocycline
(minimum effect), D- penicillamine(rarely used), Mtx if
no ILD,MMF when associated ILD.
 Raynauds-
 Avoidance of cold, frequent warming of hand and feet, stop
smoking
 Medical management – calcium channel blockers( Nifedipine
30mg bd, Amlodipine 2.5-10 mg daily)
 If ulceration present –Sildenafil, tadalafil, Prazocin, losartan,
bosentan
 Cutaneous ulcer- as discussed with raynauds; low dose
aspirin or clopidogrel, IV prostanoid ( e.g.
epoprostenol),Hydrocolloid dressing, IV iloprost
 Calcinosis cutis- low dose warfarin, Ca channel
blocker, sodium thiosulfate,
Bisphosphonate,intralesional steroid,Surgery
 Treatment of systemic involvement

Lungs
 ILD- Immunosuppressive
 PAH- Oxygen, bosentan, sildenafil, epoprostenol)
Kidney
 Renal crisis and hypertension- ACE inhibitors but not
always helpful
CVS- ACE inhibitor
GI-
 Esophageal dysmotility – Proton pump inhibitors
 Small bowel involvement-Promotility
agent( ondansetron)
 Scleroderma renal crisis

Risk factor- Early diffuse cutaneous systemic

sclerosis, Glucocorticoid use

Presentation- accelerated hypertension and

progressive renal impairment

Treatment- ACE inhibitor

2/3 patient require renal replacement

therapy
New classification criteria for SSc
Define and differentiate clinical phenotypes with
appropriate clinical examination and laboratory
testing
Able to recognize the clinical feature
 Cutaneous
 Vascular
 Gastrointestinal
 Pulmonary

Able to understand underlying pathology and

pathophysiology of renal crisis
Thank you…