1.
A 55 year old woman presents with dysphagia, diarrhea, and weight loss. Physical examination reveals a woman who appears younger than her stated age with abnormal breath sound. Lab data is significant for antibodies against DNA topoisomerase I. What is the most likely diagnosis?
Correct Answer
A. Systemic sclerosis
Explanation
The most likely diagnosis in this case is systemic sclerosis. The patient's symptoms of dysphagia, diarrhea, and weight loss, along with the presence of antibodies against DNA topoisomerase I, are consistent with systemic sclerosis. Additionally, the abnormal breath sounds may be indicative of lung involvement, which is common in systemic sclerosis. Sjogren's syndrome, CREST syndrome, discoid lupus, and systemic lupus erythematosus do not typically present with all of the symptoms and findings described in the patient.
2.
This immunofluorescent staining pattern is MOST CHARACTERISTIC of
Correct Answer
E. Systemic lupus erythematosus (SLE)
3.
This 53-year-old woman has pain and color changes in the digits of both hands. She reports difficulty breathing and in swallowing liquids and solids. Physical examination shows limited movement of the chest wall on inspiration. On auscultation, inspiratory crackles are heard at both lung bases. Which clinical laboratory test is MOST LIKELY to be positive?
Correct Answer
E. Serum antinuclear antibody (ANA)
Explanation
Topoisomerase is the specific test but it is not given above; Therefore, ANA is elevated in most of the AI diseases.
• Chronic disease characterized by:
1) chronic inflammation thought to be the result of autoimmunity
2) widespread damage to small blood vessels
3) progressive interstitial & perivascular fibrosis in skin & organs
• Skin is most commonly affected
• Gastrointestinal tract, kidneys, heart, muscles, & lungs also involved
• Two major categories:
1) diffuse scleroderma
-widespread skin involvement at onset
- with rapid progression and early visceral involvement
2) limited scleroderma
-skin involvement confined to fingers, forearms & face
.
Occurs at 50-60 yrs, F:M 3:1
• Localized or Generalized disease
• Genetics
 HLA class II genes
 Mutation of fibrillin-1 and other extracellular matrix protein gene
 Associated Antibodies:
 Anti-Scl70 (Anti-topoisomerase I)
 Anti-centromere antibody (CREST syndrome)
4.
A 55-year-old woman comes to your office complaining of dry eyes and dry mouth. Physical examination reveals a woman who appears younger than her stated age with enlarged parotid glands. Her anti-nuclear antibody test is positive. Which of the following would be most helpful in confirming your diagnosis?
Correct Answer
E. SS-A antibodies
5.
The findings seen in the gross photograph below are MOST CHARACTERISTIC of
Correct Answer
E. Systemic lupus erythematosus (SLE)
6.
A 40 Year old patient presents with shortness of breath and a skin rash. On questioning she admits that she gets fatigued with very little exertion. She has pain in her right knee, wrist joints and small joints in her fingers. She has oral ulcers and difficulty opening her mouth as her jaw hurts. She gives history of what appears to be Reynaud’s phenomenon. On examination the liver is mildly enlarged and she has a mild icterus. Both Rheumatoid factor and ANA are positive. Serum complement, C-reactive protein (CRP), SS-A antibodies, Scl-70 antibodies and anti-dsDNA antibody test is awaited. X-ray of the hands, wrist and knee joints shows non-erosive arthritis. The skin biopsy shows vasculitis, but no “onion skinning” of vessels or dermal fibrosis. The immunofluorescence report is pending. A liver biopsy report is also pending. What is the most likely diagnosis in this patient?
Correct Answer
A. Systemic lupus erythematosus
Explanation
Non-erosive arthritis is SLE, "Onion-skinning" is Scleroderma
Oral ulcers is SLE
7.
This kidney biopsy was taken from a patient who had a kidney transplant. What is the most likely diagnosis?
Correct Answer
B. Chronic rejection
Explanation
The most likely diagnosis for the kidney biopsy from a patient who had a kidney transplant is chronic rejection. Chronic rejection is a long-term process that occurs months to years after transplantation, characterized by progressive fibrosis and scarring of the transplanted kidney. It is typically associated with persistent inflammation and immune response against the transplanted organ. This can lead to a decline in kidney function over time.
8.
A 45 year old woman presents with increasing creatinine and BUN levels, 6 years after a renal transplant. A biopsy is done and shows the findings presented in Figure 1. Which of the following is the diagnosis for this patient’s condition?
Correct Answer
E. Chronic rejection
Explanation
The correct answer is Chronic rejection. Chronic rejection refers to the gradual and progressive loss of renal function over time in a transplanted kidney. The biopsy findings presented in Figure 1 are consistent with chronic rejection, showing interstitial fibrosis and tubular atrophy, which are characteristic features of this condition. This is further supported by the patient's history of increasing creatinine and BUN levels, which indicate declining kidney function. Hyperacute rejection occurs immediately after transplantation, acute rejection occurs within the first few months, graft vs host disease is typically seen in bone marrow transplants, and infection would present with different histological findings.
9.
Production of auto-antibodies against self antigens causes a number of autoimmune disorders. Which of the following hypersensitivity reactions is responsible for the disease shown in Figure 2?
Correct Answer
A. Hypersensitivity reaction type II
Explanation
Production of auto-antibodies against self antigens is characteristic of hypersensitivity reaction type II. In this type of reaction, antibodies bind to antigens on the surface of cells or tissues, leading to their destruction through various mechanisms such as complement activation or antibody-dependent cell-mediated cytotoxicity. This immune response against self antigens is responsible for the development of autoimmune disorders.
10.
This kidney biopsy was taken from a patient who had a kidney transplant. What is the most likely diagnosis?
Correct Answer
B. Chronic rejection
Explanation
The most likely diagnosis for the kidney biopsy taken from a patient who had a kidney transplant is chronic rejection. Chronic rejection is a long-term process that occurs over months or years after a transplant. It is characterized by progressive damage to the transplanted kidney, leading to a decline in kidney function. This is different from hyperacute rejection, which occurs immediately after transplantation, and acute humoral and cellular rejection, which are more acute and reversible processes.
11.
A 45-year-old man comes to your office complaining of blood in his sputum and urine. Physical examination reveals a man with hypertension, respiratory distress and edema. Urinalysis shows the presence of red blood cells and proteins in the urine. What type of hypersensitivity reaction is most likely associated with this patient’s diagnosis?
Correct Answer
C. Type II
Explanation
Based on the symptoms described, the presence of blood in the sputum and urine, along with hypertension, respiratory distress, and edema, suggests that the patient may have a condition called Goodpasture syndrome. Goodpasture syndrome is characterized by the presence of antibodies that target the basement membrane of the lungs and kidneys, leading to damage and inflammation. This type of immune response is associated with Type II hypersensitivity reactions, where antibodies bind to antigens on the patient's own cells, triggering their destruction. Therefore, the correct answer is Type II.