Quiz 3

(Ref: Harrison 20th edition, p 2700; Harrison's 18th edition, ch. 319)
 Fatigue and serous cavity effusions is a feature of myxedema heart. Myxedema would lead to carpal tunnel syndrome and is also associated with macrocytic anemia.
 In SLE the anemia is autoimmune hemolytic anemia and this is a normocytic normochromic anemia while in the question the anemia mentioned is macrocytic anemia
 Carpal tunnel syndrome is not mentioned in features of SLE.
 The classic presentation of a triad of fever, joint pain, and rash in a woman of childbearing age should prompt investigation into the diagnosis of SLE.

(Ref: Nelson 20th edition, page 1177)
 Pediatric lupus contributes to 20% of all case load of SLE. Children present with more acute illness and have more frequent hematologic and central nervous system involvement at the time of diagnosis compared to adults with SLE.
 Almost all children require corticosteroids during the course of their disease, and many are treated with immunosuppressive drugs. Mortality rates remain higher with pediatric SLE compared to adult onset SLE.
 When directly comparing pediatric SLE to adult SLE the inflammatory rashes, including the typical malar erythema, are significantly more frequent in children
than adults. Isolated discoid lupus erythematosus (DLE) is uncommon in childhood. Since choice ‘a’ mentions the word skin pigmentation and not skin rash it has been ruled out.
 Gender issues with SLE
1. During the first decade of life to 4:1
2. During the second decade to 9:1
3. Decreases to 5:1 in SLE commencing after the age of 50 year

(Ref: Harrison 20th edition, p 2606; http://www.aafp.org/ afp/2004/0715/p312.html; Harrison 19th edition, p 2205)
Acute sarcoidosis is more common in whites than in blacks and usually is associated with spontaneous remission within two years. Spontaneous remission also occurs in patients with Lofgren’s syndrome, which consists of bilateral hilar lymphadenopathy, ankle arthritis, erythema nodosum, fever, myalgia, and weight loss

(Ref: Harrison 19th edition, p 2208)
Garland’s triad (also known as the 1-2-3 sign or Pawnbrokers sign) is a lymph node enlargement pattern which has been described in sarcoidosis. It comprises of:
1. Right paratracheal nodes
2. Right hilar nodes
3. Left hilar nodes

(Ref: Harrison 20th edition, p 2578-2579; Harrison 19th edition, p2184)
Wegeners granulomatosis/ granulomatosis with angitiis is characterized by
1. Upper respiratory tract symptoms- mentioned as maxillary sinusitis
2. Lower respiratory tract symptoms-mentioned as hemoptysis
3. Kidney involved — mentioned as hematuria
4. Skin involvement- mentioned as purpura
Clinical features of Granulomatosis with angitis
1. Chronic sinusitis is the most common initial complaint in GPA, occurring in 67% of cases; failure to respond to conventional treatment is suggestive. Other ENT manifestations are as follows: Rhinitis (22%)/Epistaxis (11%)/Serous otitis media and hearing loss/strawberry gingival hyperplasia
2. Pulmonary disease may cause any of the following: Pulmonary infiltrates (71%)/Cough (34%)/Hemoptysis (18%)
3. Musculoskeletal manifestations: Myalgias/Arthralgias, usually polyarticular and symmetrical, affecting small and medium joints
4. Renal manifestations-Crescentic necrotizing glomeru-lonephritis characterized by urinary sediment with more than 5 RBCs per HPF or erythrocyte casts
5. Nervous system manifestations-Mononeuritis multiplex/ Sensorimotor polyneuropathy/Cranial nerve palsies
6. CNS manifestations include vasculitis of small to medium sized vessels of the brain or spinal cord and granulomatous masses that involve the orbit, optic nerve, meninges, or brain.
7. Cutaneous manifestations: Palpable purpura or skin ulcers (45%); ulcerations may resemble pyoderma gangrenosum

(Ref: Rheumatology E-Book, 6th edition, p 168)
RS3PE, Relapsing, seronegative, symmetric synovitis with pitting edema is defined as seronegative arthritis with the following clinical features
1. Clear pitting edema of hands (called boxing glove appearance of hands)
2. Polyarthritis with acute onset
3. Age > 50 years
4. Negative rheumatoid factor
It was earlier considered to be a subset of rheumatoid arthritis but is now regarded a different disease entity. This is because unlike rheumatoid arthritis there is absence of joint erosions and good response to steroids.