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Difficulty: MediumRheumatology and Immunology

Connect the following autoimmune and rheumatologic disorders to their characteristic, potentially severe extra-articular complications.

  • Rheumatoid arthritisCervical spine instability that can progress to compressive myelopathy
  • Ankylosing spondylitisAortitis leading to aortic root dilation and valvular regurgitation
  • Sjögren syndromeIncreased risk of B-cell non-Hodgkin lymphoma in the salivary glands
  • Systemic sclerosis (diffuse cutaneous)Oliguric acute renal failure accompanied by malignant hypertension

Answer

Rheumatoid arthritis matches with cervical spine instability; Ankylosing spondylitis matches with aortic root dilation; Sjögren syndrome matches with B-cell non-Hodgkin lymphoma; Systemic sclerosis matches with oliguric acute renal failure and malignant hypertension.
Each rheumatologic condition possesses a distinct pathogenesis that dictates its extra-articular complications. Rheumatoid arthritis causes erosive pannus in the cervical spine. Ankylosing spondylitis involves enthesitis and inflammation that can affect the aortic root. Sjögren syndrome's chronic B-cell stimulation predisposes to lymphoma. Systemic sclerosis causes vascular intimal proliferation leading to renal crisis.

Step-by-Step Solution

1
Analyze Rheumatoid arthritis (RA) for major extra-articular complications.
RA characteristically affects the cervical spine, leading to atlantoaxial subluxation and potential compressive myelopathy.
Inflammatory pannus degrades the transverse ligament of the atlas.
2
Evaluate Ankylosing spondylitis (AS).
AS is a seronegative spondyloarthropathy that can cause inflammation of the aortic root, leading to aortic regurgitation.
Extraskeletal manifestations of AS prominently include anterior uveitis and cardiovascular issues like aortitis.
3
Identify the primary severe risk in Sjögren syndrome.
Patients with Sjögren syndrome have a significantly elevated risk of developing B-cell lymphomas, particularly MALT lymphoma of the salivary glands.
Chronic inflammation and B-cell hyperactivation in exocrine glands predispose to malignant transformation over time.
4
Determine the life-threatening complication of Systemic sclerosis.
Diffuse systemic sclerosis is highly associated with scleroderma renal crisis, presenting as acute kidney injury and malignant hypertension.
Vascular damage and intimal thickening in renal arteries lead to severe ischemia and renin-angiotensin-aldosterone system activation.

Key Concept

Extra-articular complications of systemic rheumatologic diseases
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Topics

Rheumatology and Immunology

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