Mucocutaneous Disease Treatment Options

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Questions and Answers

What type of disease is being referred to in the context?

Mucocutaneous disease

What is the first-line treatment for mucocutaneous disease?

Colchicine

What is an alternative treatment option for mucocutaneous disease?

Topical steroids

What is a common symptom of mucocutaneous disease?

All of the above Signup and view all the answers

What is the primary site of mucocutaneous disease?

Both skin and mucous membrane Signup and view all the answers

What is the treatment option for more severe cases of refractory disease?

TNF inhibitors Signup and view all the answers

What is the name of the disease characterized by recurrent episodes of fever with serosal, synovial, or cutaneous inflammation?

Familial Mediterranean fever Signup and view all the answers

What is used to treat CNS disease and uveitis?

Cyclophosphomide Signup and view all the answers

What is known as Recurrent polyserositis?

Familial Mediterranean fever Signup and view all the answers

What is NOT a treatment option for refractory disease?

Methotrexate Signup and view all the answers

At what age do 90% of patients experience their first febrile episode?

By age 20 Signup and view all the answers

What is the typical duration of a febrile episode in this disease?

1-4 days Signup and view all the answers

What percentage of patients experience febrile episodes?

90% Signup and view all the answers

When do febrile episodes typically begin in this disease?

In early infancy Signup and view all the answers

What triggers febrile episodes in this disease?

Unknown triggers Signup and view all the answers

What is the most common side effect of colchicine?

Bloating, abdominal cramps, and diarrhea Signup and view all the answers

Which of the following is a serious side effect of colchicine?

Acute renal failure Signup and view all the answers

What is a common symptom of colchicine-induced neuromyopathy?

Proximal muscle weakness Signup and view all the answers

What is a laboratory finding that may be associated with colchicine-induced neuromyopathy?

Elevated creatinine kinase Signup and view all the answers

What is a serious muscle-related side effect of colchicine?

Rhabdomyolysis Signup and view all the answers

What are the two tracts through which colchicine and its metabolites are excreted?

Urinary and biliary tracts Signup and view all the answers

What is a potential side effect of using colchicine?

Neuropathy Signup and view all the answers

What is the term for a decrease in bone marrow activity, leading to decreased blood cell production?

Myelosuppression Signup and view all the answers

What two systems are affected by myelosuppression?

Hematopoietic and immune systems Signup and view all the answers

What is the result of decreased bone marrow activity?

Decreased blood cell production Signup and view all the answers

Study Notes

Mucocutaneous Disease Treatment

First-line treatment: Colchicine
Topical steroids can be tried as an alternative

Refractory Disease Treatment

Low dose systemic steroids
Azathioprine
TNF inhibitors

CNS Disease and Uveitis Treatment

High dose systemic steroids
Cyclophosphamide

Familial Mediterranean Fever (FMF)

Also known as Recurrent polyserositis
Characterized by recurrent episodes of fever with serosal, synovial, or cutaneous inflammation
Autosomal recessive disease

Clinical Manifestations of FMF

Febrile episodes that may begin in early infancy
90% of patients have their first attack by age 20
Attacks typically last 1-4 days

Colchicine

Excreted through urinary and biliary tracts
Side effects:
- Common: Bloating, abdominal cramps, diarrhea, nausea, and vomiting
- Serious: Bone marrow suppression, acute renal failure, rhabdomyolysis, and neuromyopathy (proximal muscle weakness and elevation of creatinine kinase)