Saturday, March 24, 2018

Mixed Connective Tissue Disease : An Overview

Hi everyone ! Just a short post reviewing MCTD! References are Harrison's and Medscape!

Mixed Connective Tissue Disease (MCTD)

1. What is it ?
- It's a somewhat ambiguously used term for disease characterised by a collection of few symptoms from different autoimmune connective tissue disorders.
- Namely , features of Systemic Sclerosis (SSc) , Lupus , Myositis and sometimes RA are present in some proportion in the same patient.
2. What are its chief presenting features ?
A. Features of SSc :
- Raynaud's is often the presenting feature. May also get edematous fingers.
- Dactylitis and digital gangrenes may be + due to Raynaud's
- Sclerodactyly
- Other Limited Cutaneous SSc features like CREST.

B. Features of Lupus :
- Arthritis
- Photosensitivity and Malar rash
- Evidence of Anti phospholipid Antibody Syndrome - associated with SLE. 

C. Features of Myositis :
- Proximal myopathy features
- Muscle tenderness
- May get Cutaneous features of Dermatomyositis.
Also may have Pulmonary Hypertension, Pulmonary Fibrosis.
3. What are the criteria for diagnosis ?
• Immunologically = Anti U1 RNP +

• Clinically = ( any 3 )
Mnemonic = A REM Sleep
Edematous Hands
Myositis (proven)
4. When to suspect MCTD?
- When a Patient comes with features of Limited Scleroderma (Raynaud's) , but not enough to fulfill its criteria ;

- SSc specific antibodies are not generally positive. (Anti Histone or Topoisomerase) ;

- Suspected SSc patients with  unusually prominent features of Arthritis, Muscle Pain or Rash
5.What tests would one order if suspecting MCTD ?
A. CBC with ESR -
May see Leucopenia with fairly elevated ESR

B. Serology -
• Confirm absence of SSc - Anti Centromere and Anti Topoisomerase.

• For MCTD -
U1 RNP Ab's are fairly specific

• For Myositis -
Anti Jo Ab's (Especially polymyositis)
Anti Mi Ab's (Especially Dermatomyositis)

• For Lupus -
Complement levels.

• For RA -
Anti CCP Ab's

C. Blood profile -
• Creatine Kinase - Elevated in Myositis
• Urine Routine + Microscopy for Lupus Nephritis type changes
• Electrolytes

D. For Complications -
• Chest X Ray for any ILD or fibrosis
• HRCT if needed.
• Pulmonary Function Tests
• MRI Brain for multi infarct lesions if APLA is + and if neurological changes are +
• ECG - For myocarditis
6. How is the treatment like ?
- NSAIDs - Symptomatic Relief.
- Steroids confer some Relief unlike in SSc. So it's important to differentiate the two!
- Hydroxy chloroquine and Methotrexate may be used to keep disease activity in check.
- Treatment of complications.

Hope this was helpful!
Happy Studying!
Stay Awesome!
~ A.P.Burkholderia

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