Small vessel vasculitis MCQs

Areas with low V/Q ratios produce hypoxemia or a decreased PaO2, which leads to A decrease in the dissolved oxygen content of the blood and A decrease in PaCO2, due to stimulation of the peripheral chemoreceptors. At lower PaO2 levels, arterial oxygen saturation is decreased, which decreases the oxyhemoglobin content. Because the mixed alveolar PO2 is normal and the arterial PO2 is less than normal, the A-a gradient is greater than normal. Reference: Levitzky, pp 113-116, 122-123.

HCC usually occurs after three decades of disease. And occurs exclusively in patients with cirrhosis. 50-70% - Develops chronic HCV 20-25% - Develops Cirrhosis. 1-4% - Develops HCC Reference: Harrison's Principles of Internal medicine, 21st edition page Ch: 341 Pg 2615

A unique form of hemolytic anemia (with spur cells and acanthocytes) called Zieve's syndrome can occur in patients with severe alcoholic hepatitis. Platelet counts are often reduced early in the disease, reflective of portal hypertension with hypersplenism. Serum total bilirubin can be normal or elevated with advanced disease. Prothrombin times are often prolonged and usually do not respond to administration of parenteral vitamin K. Serum sodium levels are usually normal unless patients have ascites and then can be depressed, largely due to ingestion of excess free water. Transient hyperlipidemia may also be seen. Reference : Harrison's Principles of internal medicine 21st ed chap 344 pg. 2625

The likely diagnosis is Wilson's disease. Most patients who present with neuropsychiatric manifestations have cirrhosis. The most common presenting neurologic feature is asymmetric tremor, occurring in approximately half of individuals with Wilson disease. Frequent early symptoms include difficulty speaking, excessive salivation, ataxia, masklike facies, clumsiness with the hands, and personality changes. The disease is autosomal recessive. Zinc is used for treatment of Wilson disease. Reference: Harrison's Principles of Internal Medicine 21 st Ed, Ch: 415Pg: 3236

Patient here presents with cutaneous symptoms and pulmonary involvement and histologically shows fibrinoid necrosis without granulomas. Among the small vessel vasculitis, Microscopic polyangiitis presents lacks granulomas and presents with fibrinoid necrosis. Among the given potions PAN also presents with fibrinoid necrosis without granulomas. So PAN is also a possibility. But what makes PAN unlikely is the pulmonary involvement. Reference: Harrison’s Principles of Internal Medicine 21 st edition Ch:363 Pg:2809

PAN like vasculitis is seen with Hepatitis B, C, Adenosine deaminase deficiency Type 2 and Hairy cell leukemia. PAN is a disease of middle aged, and is common in males. If a very young boy presents with PAN like symptoms Adenosine deaminase deficiency should be suspected. Reference: Harrison’s Principles of Internal Medicine, 21 st ed, Ch: 363, Pg 2810-2811

The history is suggestive of gastro oesophageal reflux for the past 10 years. Continuous acid reflux causes metaplasia of the stratified squamous epithelium (which normally lines the distal oesophagus) to an abnormal columnar epithelium. This is known as Barrett's oesophagus . Barrett's oesophagus increases the risk of development of adenocarcinoma of the oesophagus . Reference : Harrison's Principles of Internal Medicine, 21 st ed, Ch: 323 Pg: 2430

The presence of hematuria and proteinuria indicates renal involvement in the form of a glomerulonephritis. Glomerulonephritis along with upper respiratory tract involvement in the form of recurrent sinusitis and pulmonary involvement indicates a small vessel vasculitis- Wegener’s Granulomatosis. 90% of patients Wegener’s granulomatosis (Granulomatosis with polyangiitis) is associated with c ANCA positivity directed against anti proteinase 3. Positive ANA may be seen in a variety of collagen vascular diseases especially SLE. Positive Kveim test is seen in Sarcoidosis Positive ASO titre is seen in Acute Rheumatic fever. Reference: Harrison’s Principles of Internal Medicine 21 st edition Ch:363 Pg:2806