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Author: Robert A. Kalish, M.D.

Case 1

A 72 year old man was seen in the office because of severe right temporal headache and malaise. This had developed insidiously over the last three weeks, and was associated with fever and a three pound weight loss. He also had mandibular pain while chewing. Several months earlier, he had severe morning stiffness affecting his shoulders and hips.

On examination he had a low grade fever and the general examination showed a right temporal artery which was indurated, tender, and quite prominent. He had a normal joint examination.

Laboratory studies showed a normochromic, normocytic anemia, an ESR of 120 mm/hr, and no hematuria.

  1. Which diagnosis comes to mind and how is it made?
  2. Was the patient's joint pain due to fibromyalgia? Why or why not?
  3. What are the most serious complications of the disease?
  4. What are the pathognomonic features of the histopathology of this disease?

Case 2

An eight year old boy came to his pediatrician because of recurrent belly pain and a lower extremity rash. A few weeks earlier, he had a viral syndrome characterized by cough and fever. He was treated with trimethoprim-sulfamethoxazole and the infection resolved.

The belly pain was intermittent, and the rash was located over the shins and was purpuric.

On examination, he had no fever, murmur, organomegaly, or synovitis. Palpable purpura was noted over the legs. The abdomen was tender, with some peritoneal findings. The stool tested positive for occult blood.

Laboratory tests showed a white count of 13,000 with 60% neutrophils and no bands, a microcytic anemia, a platelet count of 200,000 and an ESR of 58 mm/hr.

  1. Which vasculitis comes to mind? Which vessel size is affected?
  2. Describe the pathologic findings in this disease.
  3. Discuss the laboratory findings.

Case 3

A 32 year old woman presented with polyarthralgias and fatigue. She had morning stiffness which lasted several hours. She had no skin rashes. Sometimes her fingers would turn white on cold exposure. Low grade fevers recently developed.

Physical examination showed a young woman with an oral temperature of 100 degrees Fahrenheit. The blood pressure was 170/100 in the right arm. She had tenderness and swelling in the proximal interphalangeal joints of both hands and bilateral knee effusions. The skin over the fingers was tight, and shiny and she had difficulty making a fist. Rales were heard at the bases of both lungs. She had a normal cardiac examination with the exception of a fast rate.

Laboratory tests available at the time of her visit were:

HCT 38
WCB 4700 with a normal differential
PLT 290,000
CREAT 1.8 mg/dl
U/A mild proteinuria
ANA 1:2560 in a nucleolar pattern
RF negative
  1. What features support the diagnosis of lupus? Does this patient have scleroderma?
  2. Which serological tests would help confirm lupus? Scleroderma?
  3. Do the renal findings help distinguish the cause of the patient's findings? What are the renal manifestations of lupus? Of scleroderma?
  4. What clinical test is important to do given the patient's history of the fingers becoming white upon exposure to the cold?
  5. Review the histopathologic features of lupus nephritis and scleroderma renal crisis.

Case 4

A 35 year old woman was admitted for evaluation of bloody rhinorrhea, recurrent ear aches, polyarthralgias, and cough productive of bloody sputum. Fever was present, along with fatigue. These symptoms had progressed over several weeks and she finally sought medical attention.

On examination, she had a collapsed nasal bridge and bloody secretions in both nares. Lung examination showed abnormal breath sounds at both bases, and egophany. The joints all moved well, and no skin rashes were noted.

Laboratory studies showed a normochromic normocytic anemia, ESR of 100 mm/hr, creatinine of 2.8 mg/dl, and the urinanalysis showed red blood cells. The chest xray revealed multiple pulmonary nodules, some of which were cavitated. The ANA and RF were negative.

  1. Which vasculitis comes to mind?
  2. What is an ANCA? Define c-ANCA and p-ANCA. Which of the two is this patient most likely to have?
  3. What are the pathologic features of this patient's disease?