This document discusses various topics related to dermatomyositis and systemic sclerosis including: 1. Characteristic features, differences between Gottron papules and sign, and electrophysiological features in muscle involvement of dermatomyositis. 2. Differences between limited and diffuse systemic sclerosis, causes of renal crisis and death in systemic sclerosis, and treatments for Raynaud's phenomenon and pulmonary arterial hypertension. 3. Newer antifibrotic drugs for systemic sclerosis including tyrosine kinase inhibitors, pirfenidone, and PPAR agonists.

1. Dermatomyositis, Systemic
sclerosis
Edited by Dr Saira Zakir

2. • 1.characteristic cutaneus features of
dermatomyositis
• 2.difference between gottron papules and
gottron sign.
• 3.electrophysiological features in muscle
involvement in dermatomyositis
• 4.difference between limited and diffuse systemic
sclerosis
• 5.cause of renal crisis in systemic sclerosis
• 6.cause of death in systemic sclerosis

7. • How to treat a patient with Raynauds having
low blood pressure?
– Raynauds shd b treated with topical gtn /2%
nifedipine cream/
– Avoid ccb in hypotension
– Give arbs tab losartan 25mg at night with bp
monitoring ..can increase upto 50mg wth bp
monitringIf no improvemnt after 2wks fFlouxetine
20/day

8. • Investigations for Pulmonary arterial hypertion ? And
treatment?
– Pfts
– Fev1
– DLCO
– Ecg
– Echo
– 6min walk test
– Rt heart catherization
– MxEndothelin receptr blocker: bosentan,
– Embrisentan
– Pde5 inhibitr: sildenafil 20mg tds
– Prostacycline analogues :ilosprost

9. • Can ACE inhibitors be given in pregnant females
with Systemic sclerosis having renal involvement?
– Discontinue ace inhib as soon as pregnancy detected;
during the second and third trimesters of pregnancy,
drugs that act directly on the renin-angiotensin have
been associated with fetal injury that includes
hypotension, neonatal skull hypoplasia, anuria,
reversible or irreversible renal failure, and death
– If in renal crises
• Iv ig
– Elective termination of preg

10. • Name of antifibrotic drugs
• Antifibrotic drugs TGF Beta, tyrosine kinases
and PPAR have fibrotic role so we use drugs
against them
– 1. Tyrosine kinase inhibitors Nintedanib(ofev),
imatinib,nilotinib,dasatinib
– 2. TGF beta inhibitor Pirfenidone( Esbiet)
– 3. PPAR stimulator lanifibranor
– 4. other antifibrotics include penicillamine,
cyclophosphamide, rituximab, abatacept,
mycophenolate mofetil

11. • 1.What is antisynthetase syndrome?
– Antisynthetase antibody syndrome:Myositis arthritis, Raynaud’s
phenomenon, ILD, fever and acral skin changes, called ‘mechanic’s
hands’ (see Presentation section). ILD is particularly common,
occurring in around 75% cases ].
• Most commonly anti‐histidyl tRNA synthetase
• (anti‐Jo‐1) antibody, which is found in 10–20% of DM cases
– ii) *no increase risk of malignancy *. More risk of calcinosis *pmore
risk of lipodystrophy ass with insulin resistnce dm acanthosis nigricans
* anti jo anitbody + * anti tif ¥ is not ass with cancer as compare to
adult dms
– iii) Electromyography can confirm a myopathic process with early
recruitment of multiple, small‐amplitude, short‐duration motor
unit action potentials

12. • all old and recent .Dermatomyositis:
• .How juvenile dermatomyositis differs from
adult dermatomyositis?

13. • 1..features of juvenile dermatomyositis(dms)
• 2...pregnancy and dms
• 3...from which muscle to take biopsy from in
dms
• 4...tests for raynauds phenomenon
• 5..when to give steroids in ss
• 6..histopathology of ss

16. • 1* What is ENA
• 2* Diagnostic Crieteria for Systemic Sclerosis
• 3* Cuttoff Point between localized
Scleroderma and diffuse scleroderma
• 4*What is CADM
• 5* What are mechanistic Hands
• 6*causes of Digitate keratosis

19. • 1:Difference between primary n secondary
raynauds
• 2: nail changes in limited n disseminated systemic
sclerosis
• 3: when will you label renal crisis in systemic
sclerosis
• 4: percentage of malignancy in dermatomyositis
5: site of biopsy in dermatomyositis
• 6: difference between juvenile n adult
dermatomyositis

22. • 1. Treatment of juvenile dermatomyositis
• 2. Difference between amyopathic dermatomyositis
(CADM) and dermatomyositis.
• 3. How will u give cyclophosphamide in dermatomyositis .
• 4. Criterias of dermatomyositis and severity index of DM.
• 5. How to treat skin disease in systemic sclerosis, any new
advancement also ?
• 6. Criteria for Follow up and monitoring for skin and
pulmonary involvement.
• 7. Effect on fetus of a mother with systemic sclerosis ,
prenatal monitoring and lactation safe or not ?

27. • 1.How to differentiate b/w Proximal myopathy
due to Dermatomyositis & steroid induced
(clinically, labs, biopsy)
• 2. What is myopathy and myositis?
• 3. Wht diseases mimic systemic sclerosis and
their differentiating points?
• 4. Role of environment/ radiation in s.s?
• 5. How will you investigate and treat
cutaneous findings of systemic sclerosis?

28. • 1.2.Steroid induced myopathy >>> mild weakness, minimal
CK elevation,minimal EMG findings,mild fibrotic changes on
histology.Improves on withdrawing steroids.
• Myositis>>> improves with steroids.
• 3. Sceroderma spectrum disorders.Pansclerotic morphea/
eosinophilic fascitis (sparing of hands and feet)Scleredema
(affects upper back, neck n face)Scleromyxedema (waxy
papules, leonine facies)Nephrogenic systemic fibrosis (lower
limbs, renal failure, gadoliniun exposure)
• 4. Silica,vinyl chloride,aromatic solvents,welding
fumes,white spirit,ketones.
• 5.investigations>>durometry, OCT, USG.Treatment...metho,
MMF, AZA.ivig,cyclosporine

29. • 1.Pathogenesis of DM.
• 2.differences between lung changes of DM
from systemic sclerosis?
• 3.difference of lung findings of SS from SLE?
• 4.What is hydrotherapy in SS?
• 5.how to assess lower esophageal sphincter
function, in SS,,which tests n their
mechanisms?
• Apply white soft paraffin on hands & dip
hands in warm water for 15mins several times
a day... It relieves skin tightening, joint pains &
Raynaud's phenomenon.. ( Ref: Dr Tariq
Rashid notes)

36. • How will you differentiate myopathy of DMS
vs steroid induced myopathy?
• How will u differentiate rash of sle from DMS ?
Factors associated with malignancy in DM?
• How will u reduce skin tightness in
scleroderma What are the bony changes in
scleroderma ?

37. • 2:in SLE rash over hands characteristically
spares meta carpophalangeal and
interphalangyeal joints but it is involved in
dermatomyositis.
• 3::uncreasing age,,severe skindisease with
necrosis,,dysphagia,,lack of extramuscular
systemic features(ILD),,Antibody to anti-TIF1
or NXP-2
• 4::d-penicillamin 125mg on alternate
days,,cyclosporine,tacrolimus,IVIG,,infliximab,i
sotretinoin 1mg/kg,UVA
• 5::thickening of tendon retinacula of finger
flexors and extensors of wrist,elbow,knees and
ankle

39. • 1.Difference between polymyositis and
dermatomyositis
• 2. When will u screen for malignancy in a
patient with dermatomyositis
• 3. Antibody ass with juvenile dm
• 4. Diff between mrss and modified mess
• 5. Malignancy ass with systemic sclerosis
• 6. Which antibody has better prognosis

42. • How to differentiate amyopathic DM from
treatment modified DM
• Points to ask in history of Raynaud's
phenomenon
• Causes of sclerodactyly Newer advancements
in treatment of scleroderma renal crisis

44. • 6. What is normal mouth aperture size? When do we call it microstomia ?
How do we measure it?
– Normal mouth opening is 35 to 55 mmMouth opening less than 35 mm is
microstomiaNormal mouth opening is the distance between the incisal edge
of maxillary central incisor and the incisal edge of mandibular central incisor
when the mouth is opened as wide as possible without any difficulty
• 7. D/Ds of microstomia?
• Trauma
• Ingestion of caustic substances
• Electrical and chemical burns
• Scleroderma
• Epidermolysis bullosa
• Holoprosencephaly
• Richieri-Costa-Pereira syndrome
Freeman-Sheldon syndrome
• Hallerman-Streiff syndrome
• Hutchinson-Gilford
• Progeria Burton
• skeletal dysplasia
• Fine-Lubinsky syndrome Leopard syndrome
• Auriculo-condylar syndrome Digeorge syndrome

45. • Q1. Why patient of SYSTEMIC SCLEROSIS LOOSES
WEIGHT?
• Q: 2 what's mask like face? WHY IT OCCURRS IN
SYSTEMIC SCLETOSIS? D/ D OF MASK LIKE FACE?
• Q: 3 Why systemic sclerosis patient develops
anemia?DM QUESTIONQ
• 4: D/ D OF HELITROPE RASH?
• Q5: DIAGNOSTIC CRITERIA FOR DM?
• Q: 6 DM SINE DERMATOMYOSITIS?

49. • 1.. What is differnce between joints of systemic
sclerosis and sle joint and rhematoid arthritis joint
– SLE: arthralgia more common than arthritis, non erosive
type arthritis seen,
– RA like joints termed rhupusRA: MCP and PIP involved,
tenosynovitis more often seen and typical serologySS: only
joint pains plus minus mild swelling is seen without Frank
arthritis
• 2..how will u see clinically that pt had developed
pulmonary hypertention
– 2. Hepatomegaly, ascites (due to right heart failure) and
fixed second heart sound on cardiac auscultation

50. • 1) management of cutaneous manifestation of
dermatomyositis
• 2) poor prognostic factor of dermatomyositis
• 3)drugs causing dermatomyositis
• 4)indication of steroid in systemic sclerosis
• 5)treatment of cutaneous lesion of systemic
sclerosis
• 6)infrared thermography available here or not?