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QUESTIONS FOR MULTIPLE AUTOIMMUNE DISEASES

Nattaporn Tesavibul, M.D.

Immunology Service
Massachusetts Eye & Ear Infirmary
Harvard Medical School
Boston, MA

1. Which of the following statements regarding multiple autoimmune diseases is not correct?

a. For a patient with an autoimmune disease, the likelihood of developing a second autoimmune disease is 50%.
b. Multiple autoimmune syndrome is the combination of at least three autoimmune diseases in the same patient.
c. Overlap syndrome is a term used for patients who have more than one established autoimmune disease.
d. Mixed connective tissue disease is a syndrome with features of SLE, systemic sclerosis, polymyositis and rheumatoid arthritis.

2. Which of the following statements regarding the epidemiology of ocular cicatricial pemphigoid is incorrect?

a. OCP has a female preponderance.
b. The estimated prevalence is 1 in 15,000 to 1 in 20,000.
c. This disease has a worldwide distribution and affects all races.
d. OCP is a disease of the middle age with the average age of onset at 40 years.

3. Which of the following medications has not been associated with drug induced OCP?

a. Atropine
b. Pilocarpine
c. Timolol
d. Epinephrine

4. What is the characteristic immunopathology of OCP?

a. Immunoglobulins and complement components are present in the epithelial basement membrane zone of the conjunctiva.
b. Immunofluorescence staining shows immunoglobulin deposition in the conjunctival stroma.
c. Colloid bodies are often found in the subepithelial region.
d. Fibrin deposition is found along the basement membrane zone of the conjunctiva.

5. Which of the following statements regarding the immunopathology of OCP is not correct?

a. Circulating autoantibodies to conjunctival basement membrane are seldom found in OCP patients.
b. ANA can be positive in 67% of OCP patients.
c. Circulating autoantibodies to thyroid and adrenal gland can be detected in OCP patients.
d. Serum interleukin 2 receptor (sIL-2R) can be elevated in OCP patients.

6. Which of the following statements is incorrect regarding the ocular manifestations of OCP?

a. OCP is usually bilateral.
b. The subepithelial fibrosis in OCP can be best demonstrated in the tarsal conjunctiva as fine white striae.
c. Stage 2 of OCP is characterized by fornix foreshortening.
d. Stage 3 of OCP is the untreatable end stage.

7. What is the most serious sign/symptom that physicians need to be aware of when treating OCP patients?

a. Chronic eye irritation.
b. Gingivitis.
c. Pruritic blisters at the skin.
d. Dysphagia.

8. Which of the following statements is incorrect regarding the treatment of OCP?

a. Treatment usually begins with systemic Dapsone 25 mg twice daily in a mild case.
b. The early stage of OCP can be treated with topical immunosuppressive agents alone.
c. Secondary trichiasis and tear film abnormalities should be treated.
d. Keratoplasty in OCP patients has a low probability of success.

9. Which of the following findings is not characteristic of lichen planus?

a. A violaceous, flat-topped, polygonal papule on the skin.
b. Papules are usually on the extensor surfaces of the forearms, neck, thighs, chin and lower back.
c. Lesions are usually symmetrical.
d. Mucous membrane lesions occur in about two-thirds of all cases.

10. Which of the following findings is not an immunopathologic characteristic of lichen planus?

a. Subepidermal and intraepidermal colloid bodies.
b. Broad band fibrin deposition along the basement membrane zone.
c. Multilamellar fragmented conjunctival basement membrane.
d. Generalized deposition of complement components at the dermoepidermal junction.

ANSWERS TO MULITPLE AUTOIMMUNE DISEASE QUESTIONS

1. a.

The likelihood of developing a second autoimmune disease is 20% in this type of patients.

( Humbert Ph, Dupond JL, Vuitton D, Agache P: Dermatological autoimmune diseases and the multiple autoimmune syndromes. Acta Dermato-venereo suppl 148: 1-8, 1989)

2. d

OCP is a disease of the elderly with an average age of onset of 60 years.

(Foster CS: Cicatricial pemphigoid. Tr Am Ophthalmol Soc 84: 528-530, 1986)

3. a

Pilocarpine, Timolol and Epinephrine, except Atropine, have all been associated with drug induced OCP.

(Foster CS: Cicatricial pemphigoid. Tr Am Ophthalmol Soc 84: 535, 1986 and Albert DM, Jakobiec FA (eds): Principles and Practices of Ophthalmology. Philadelphia, WB Saunders, pp 196, 1993)

4. a

(Albert DM, Jakobiec FA (eds): Principles and Practices of Ophthalmology. Philadelphia, WB Saunders, pp 198, 1993)

5. a

The autoantibodies in patients with OCP can be detected in all patients when the disease is active.

(Albert DM, Jakobiec FA (eds): Principles and Practices of Ophthalmology. Philadelphia, WB Saunders, pp 196, 1993 Foster CS: Cicatricial pemphigoid. Tr Am Ophthalmol Soc 84: 532, 1986)

6. d

Stage III OCP characterized by symblepharon formation and is neither the untreatable nor the end stage of the disease.

(Foster CS: Cicatricial pemphigoid. Tr Am Ophthalmol Soc 84: 544, 1986)

7. d

OCP can be fatal if tracheal or esophageal strictures occur.

(Foster CS: Cicatricial pemphigoid. Tr Am Ophthalmol Soc 84: 531, 1986)

8. b

OCP is a systemic disease and should be treated systemically. Details of the treatment modalities can be found in the provided reference below or any standard ophthalmology textbooks.

(Albert DM, Jakobiec FA (eds): Principles and Practices of Ophthalmology. Philadelphia, WB Saunders, pp 198-199, 1993)

9. b

Lesions usually occur on the flexor surfaces of the forearms, neck, thighs, chin and lower back.

(Fitzpatrick TB, Eisen AZ, Wolff K, Freedberg IM, Austin KF (eds): Dermatology in General Medicine. McGraw Hill, pp 656-7 889, 1979)

10 .d

a-c are all correct concerning the immunopathology of lichen planus.

(Konrad K, Pehamberger H, Holubar K: Ultrastructural localization of immunoglobulin and fibrin in lichen planus. Am Acad Dermatol 1(3): 233-239, 1979)

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