Dermatomyositis and Polymyositis diagnosis

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Questions and Answers

What is the most common pattern of peripheral microangiopathy observed in Systemic Sclerosis patients?

Dermatomyositis pattern

Scleroderma pattern (correct)

Raynaud's pattern

Mixed Connective Tissue Disease pattern

What is the purpose of the capillaroscopic skin ulceration risk index (CSURI)?

To predict the development of finger ulcerations in Systemic Sclerosis patients (correct)

To monitor the progression of Rheumatoid Arthritis

To diagnose Dermatomyositis in patients

To predict the development of internal organ involvement in Systemic Sclerosis patients

What is the significance of the 'early' pattern in Systemic Sclerosis?

It can be detected many years before full clinical manifestation of Systemic Sclerosis (correct)

It is a indication of internal organ involvement

It is a late stage of the disease

It is a characteristic of Dermatomyositis

What is the connection between the capillary image and internal organ involvement in Dermatomyositis?

The connection has not yet been fully studied Signup and view all the answers

What is the typical feature of capillaroscopy in Dermatomyositis patients?

Giant loops and ramified capillaries with intense neoangiogenesis Signup and view all the answers

What is the characteristic of capillary abnormalities in patients with polymyositis (PM)?

Rare, mild, and comparable to the healthy population Signup and view all the answers

Which of the following diseases is characterized by progressive destruction of bile ducts, cholestasis, and presence of antimitochondrial antibodies (AMA)?

Primary Biliary Cirrhosis Signup and view all the answers

What is the potential screening method for Primary Biliary Cirrhosis (PBC) patients with symptoms suggesting Systemic Sclerosis (SSc)?

Capillaroscopy Signup and view all the answers

Which of the following diseases combines symptoms of Systemic Sclerosis (SSc), Dermatomyositis (DM), rheumatoid arthritis (RA), and systemic lupus erythematosus (SLE)?

Mixed Connective Tissue Disease (MCTD) Signup and view all the answers

What is the characteristic of the clinical course of Scleromyositis (ScM)?

Usually more benign than Systemic Sclerosis Signup and view all the answers

What is the suggested pathogenetic mechanism in Dermatomyositis (DM)?

Microangiopathy Signup and view all the answers

What is the characteristic of capillaroscopic findings in Mixed Connective Tissue Disease (MCTD)?

Varying from a normal image through mild non-specific abnormalities to severe microangiopathy Signup and view all the answers

What is a common feature of nail fold capillaries in patients with SLE?

Mild changes in up to 30% of patients Signup and view all the answers

Which of the following conditions is associated with multiple microhaemorrhages and hemosiderin deposits?

Antiphospholipid syndrome Signup and view all the answers

What is the significance of scleroderma-like capillary abnormalities in patients with ILD?

They are likely to develop SSc or an associated disease Signup and view all the answers

What is a characteristic feature of nail fold capillaries in patients with RA?

Prominent, clearly visible subpapillary venous plexus Signup and view all the answers

What is the significance of capillaroscopic findings in patients with APS or vasculitis?

They should be interpreted with caution Signup and view all the answers

What is a common association with interstitial lung disease (ILD)?

Scleroderma spectrum disorders Signup and view all the answers

What is the role of capillaroscopy in equivocal cases of ILD?

It may be useful in identifying patients with scleroderma-like capillary abnormalities Signup and view all the answers

What is an important consideration when interpreting capillaroscopic findings in patients with peripheral microangiopathy?

More common medical conditions may also be a cause of peripheral microangiopathy Signup and view all the answers

Study Notes

Capillaroscopy in Connective Tissue Diseases

Capillaroscopy can be useful in differentiating DM and polymyositis (PM)
In PM, capillary abnormalities are rare, mild, and comparable to the healthy population
In mixed connective tissue disease (MCTD), capillary involvement varies greatly, from normal to severe microangiopathy

Mixed Connective Tissue Disease (MCTD)

MCTD combines symptoms of SSc, DM, rheumatoid arthritis (RA), and systemic lupus erythematosus (SLE)
Scleroderma-like abnormalities in MCTD often accompany interstitial lung disease (ILD)

Scleromyositis (ScM)

ScM is an overlap syndrome combining features of SSc and DM/PM
ScM is associated with the presence of anti-PM/Scl antibodies
Clinical course of ScM is usually more benign than SSc, with a normal capillary image

Primary Biliary Cirrhosis (PBC)

PBC is a chronic autoimmune liver disease characterized by progressive destruction of bile ducts and cholestasis
PBC is the most common liver disease in SSc patients
Capillaroscopy can be a potential screening method in PBC patients with symptoms suggesting SSc

Systemic Sclerosis (SSc)

SSc is a rare connective tissue disease presenting with diffuse fibrosis and dysfunction of internal organs due to microangiopathy
In over 95% of SSc patients, peripheral microangiopathy follows a typical scleroderma pattern
Capillaroscopy can predict development of finger ulcerations in SSc patients

Capillaroscopic Skin Ulceration Risk Index (CSURI)

CSURI is a tool devised by Sebastiani et al., based on the formula D × M/N2
CSURI correctly predicted formation of new ulcerations within 3 months following capillaroscopy, with a positive predictive value of 81% for CSURI > 2.96 and a negative predictive value of 93% for CSURI < 2.96

Dermatomyositis and Antisynthetase Syndrome

Dermatomyositis (DM) and antisynthetase syndrome are connective tissue diseases with prominent muscle and skin involvement
Presence of severe capillary abnormalities such as giant loops and ramified capillaries with intense neoangiogenesis is common in DM patients
Scleroderma-like capillary abnormalities may correlate with ILD

Other Connective Tissue Diseases

In SLE, nail fold capillaries are often normal, and mild changes occur in up to 30% of patients
In RA, patients have increased capillary tortuosity and prominent, clearly visible subpapillary venous plexus
Antiphospholipid syndrome (APS) and small-vessel vasculitides can present as multiple microhaemorrhages and hemosiderin deposits, with no morphological changes in capillaries

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Description

This quiz covers the differences in capillaroscopic findings between Dermatomyositis (DM) and Polymyositis (PM), including the autoimmune response and capillary abnormalities involved in each disease.