Rheumatology

Question 1

Antiphospholipid antibody

Answer 1

Diagnosis
1. Arterial or venous thromboembolism or pregnancy complication
2. Anti-cardiolipin, B2 glycoprotein or lupus anticoagulant confirmed three months apart on blood tests.
May be primary or secondary

Question 2

What is RNP associated with

Answer 2

MCTD

Question 3

Polyarteritis nodosa

Answer 3

Classic polyarteritis nodosa (PAN) is a systemic vasculitis characterized by necrotizing inflammatory lesions that affect medium-sized and small muscular arteries, preferentially at vessel bifurcations. [1] These lesions result in microaneurysm formation, aneurysmal rupture with hemorrhage, thrombosis, and, consequently, organ ischemia or infarction.
Lungs and kidneys are usually spared with PAN.
ACR criteria 3/10:
Weight loss of 4 kg or more
Livedo reticularis
Testicular pain/tenderness
Myalgia or leg weakness/tenderness
Mononeuropathy or polyneuropathy
Diastolic blood pressure greater than 90 mm/Hg
Elevated blood urea nitrogen (BUN) or creatinine level unrelated to dehydration or obstruction
Presence of hepatitis B surface antigen or antibody in serum
Arteriogram demonstrating aneurysms or occlusions of the visceral arteries
Presence of polymorphonuclear neutrophils in a biopsy specimen from a small- or medium-sized artery

Question 4

Microscopic polyarteritis

Answer 4

Microscopic polyangiitis (MPA; formerly called microscopic polyarteritis) is an ANCA-associated systemic vasculitis that has some features similar to those of classic PAN, with the additional involvement of renal glomeruli and pulmonary capillaries.

Question 5

Features of sarcoidosis

Answer 5

hypercalcaemia, bilateral hilar lymphadenopathy, chest symptoms. Biopsy reveals non-caseating granuloma.

Question 6

Dermatomyositis

Answer 6

purple heliotrope rash and polymyositis (proximal muscle weakness)

Question 7

ANA

Answer 7

SLE

Question 8

Anti-centromere antibodies

Answer 8

systemic sclerosis

Question 9

Anti-smooth antibodies

Answer 9

Autoimmune hepatitis

Question 10

Goodpasture Syndrome

Answer 10

Goodpasture disease is a term used to describe glomerulonephritis, with or without pulmonary hemorrhage, and the presence of circulating anti–glomerular basement membrane (anti-GBM) antibodies. At some time during the course of illness, as many as one third of patients with Goodpasture syndrome have circulating antineutrophilic cytoplasmic antibodies (ANCAs) in addition to anti-GBM antibody. In most cases, the ANCA antibodies precede the development of anti-GBM antibodies by months to years.

Question 11

Granulomatosis with polyangiitis

Answer 11

Granulomatosis with polyangiitis (GPA), formerly known as Wegener granulomatosis, is a rare multisystem autoimmune disease of unknown etiology. GPA is one of the antineutrophil cytoplasmic antibody (ANCA)–associated vasculitic disorders, which can affect the ears, nose, throat, respiratory tract, skin, kidneys, and peripheral nervous system. Eosinophilia is not a feature of GPA but rather of eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). Rheumatoid factor is positive in a low titer in two thirds of patients, whereas antinuclear antibody is present in 10-20% of patients.

Question 12

Systemic sclerosis

Answer 12

The mnemonic for the main symptoms of systemic sclerosis, also known as scleroderma, is CREST:
C: Calcinosis, or calcium deposits in the skin, usually on the fingers
R: Raynaud’s phenomenon, or spasms in the blood vessels of the fingers or toes in response to cold or stress
E: Esophageal dysmotility, or difficulty swallowing
S: Sclerodactyly, or tightening of the skin that causes the fingers to bend
T: Telangiectasia, or dilated vessels on the skin of the fingers, face, or inside of the mouth

ANA and RF may be positive.
Limited sclerodoma(avoids trunks and proximal limbs)- anti-centromere (lung mor involved-pulmonary HTN)
Diffuse scleroderma- anti-Scl70 (renal crisi more common)