Systemic connective tissue disorders involve multiple organs and organ systems beyond just the joints, muscles, and skin. There are over 200 disorders that can affect connective tissue, with causes and symptoms varying between each type. Some examples of systemic connective tissue disorders include systemic lupus erythematosus (SLE), which causes the immune system to mistakenly attack healthy tissue, dermatopolymyositis which includes skin rashes and muscle inflammation, and inherited disorders such as Ehlers-Danlos syndrome, epidermolysis bullosa, Marfan syndrome, and osteogenesis imperfecta. Risk factors include female sex hormones, sunlight, smoking, and vitamin D deficiency.

1. SYSTEMIC CONNECTIVE
TISSUE DISORDERS

2. INTRODUCTION
• Connective tissue disease refers to a group of disorders
involving the protein-rich tissue that supports organs and
other parts of the body.
• Examples of connective tissue are fat, bone, and
cartilage.
• These disorders often involve the joints, muscles,
and skin.

3. • Systemic connective tissue disorders involve other
organs and organ systems, including
the eyes, heart, lungs, kidneys, gastrointestinal tract,
and blood vessels.
• There are more than 200 disorders that affect the
connective tissue.
• Causes and specific symptoms vary by the different
types.

4. INHERITED DISORDERS
OF CONNECTIVE TISSUE
• Ehlers-Danlos syndrome (EDS)
• Epidermolysis bullosa (EB)
• Marfan syndrome
• Osteogenesis imperfecta

5. SYSTEMIC LUPUS
ERYTHEMATOSUS
• Systemic lupus erythematosus (SLE), also known simply
as lupus, is an autoimmune disease in which the
body's immune system mistakenly attacks healthy tissue
in many parts of the body.

6. Signs&symptoms
• Painful and swollen joints
• Fever
• Chest pain
• Hair loss
• Mouth ulcers
• Swollen lymph nodes
• Feeling tired
• Red rash

7. CAUSES
• Hormonal
• Environmental
• Genetic factors

8. RISK FACTORS
• Female sex hormones
• Sunlight
• Smoking
• vitamin D deficiency
• Infections

9. • Dermatopolymyositis is a family of myositis disorders
that includes polymyositis and dermatomyositis.
• Juvenile dermatomyositis (JDM) is a disease in children
that causes skin rash (dermato) and muscle
inflammation (myositis), resulting in weak muscles.

10. ICD-10-CM GUIDELINES
• Polyarteritis nodosa and related conditions-M3O
• Other necrotizing vasculopathies-M31
• Systemic lupus erythematosus-M32
• Dermatopolymyositis-M33

11. • Systemic sclerosis-M34
• Other systemic involvement of connective tissue-M35
• Systemic disorders of connective tissue in diseases
classified elsewhere- M36
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12. THANK YOU