Insulin-resistance type B

Type B insulin-resistance syndrome belongs to the group of extreme insulin-resistance syndromes (which includes leprechaunism, the lipodystrophies, Rabson-Mendenhall syndrome, and type A insulin resistance syndrome;and occurs in the context of immune dysfunction.

Epidemiology

It is a rare disorder that affects middle-aged adults, predominantly females.

Cause

The syndrome is associated with the presence of serum auto-antibodies against the insulin receptor.

Signs and symptoms

It may occur in the context of a well-characterized autoimmune disease (systemic lupus erythematosus; ), or suggest an immune disease (such as an elevated sedimentation rate, proteinuria, high levels of antinuclear antibodies or decreased levels of certain complement factors). The onset of the disease is usually marked with a rapidly progressive nonketotic and severely insulin-resistant diabetes, along with acanthosis nigricans (the typical skin lesion associated with insulin resistance) and hirsutism. Paradoxal hypoglycemia is sometimes observed and may be extremely severe.

For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. 80%-99% of people have these symptoms

• Abnormal circulating fatty-acid concentration
• Abnormal oral glucose tolerance
• Acanthosis nigricans(Darkened and thickened skin)
• Elevated erythrocyte sedimentation rate(High ESR)
• Fasting hyperinsulinemia(High blood insulin levels while fasting)
• Hypotriglyceridemia(Low blood triglyceride levels)
• Postprandial hyperglycemia
• Systemic lupus erythematosus
• Weight loss

30%-79% of people have these symptoms

• Abnormality of circulating leptin level
• Antinuclear antibody positivity
• Decreased serum complement factor B
• Enlarged polycystic ovaries(Enlarged ovaries with cysts)
• Fatigue(Tired)
• Glycosuria(Glucose in urine)
• Hirsutism(Excessive hairiness)
• Hypoalbuminemia(Low blood albumin)
• Increased serum testosterone level
• Insulin-resistant diabetes mellitus(Insulin resistant diabetes)
• Leukopenia(Decreased blood leukocyte number)
• Nephritis(Kidney inflammation)
• Proteinuria(High urine protein levels)
• Type II diabetes mellitus(Noninsulin-dependent diabetes)

5%-29% of people have these symptoms

• Abnormal salivary gland morphology(Abnormality of the salivary glands)
• Alopecia(Hair loss)
• Biliary cirrhosis
• Diabetic ketoacidosis
• Fasting hypoglycemia(Low blood sugar when fasting)
• Hodgkin lymphoma
• Hyperinsulinemic hypoglycemia
• Increased body weight
• Increased circulating IgA level
• Increased circulating IgG level
• Multiple myeloma
• Osteoarthritis(Degenerative joint disease)
• Pneumonia
• Skin rash
• Thrombocytopenia(Low platelet count)

Diagnosis

The diagnosis is based on the clinical picture, results of laboratory tests, and on detection of anti-insulin receptor auto-antibodies in the serum.

Management and treatment

Treatment of the underlying autoimmune disease consists of non-specific immunosuppressors associated with very high doses of insulin to try to control the hyperglycemia.

Prognosis

Prognosis depends on the underlying autoimmune disease, but it is unfavorable in cases with hypoglycemia (leading to death in 50% of cases).

NIH genetic and rare disease info

• NIH info on Insulin-resistance type B

Insulin-resistance type B is a rare disease.

Insulin-resistance type B Resources
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