Examkin

Question

Difficulty: MediumImmunologic and Inflammatory Processes

Match each patient's clinical presentation with the autoantibody most specifically associated with their underlying immunologic pathology.

  • A 28-year-old woman with a photosensitive malar rash, inflammatory arthritis, and new-onset nephrotic-range proteinuria.Anti-double-stranded DNA (anti-dsDNA) antibodies
  • A 45-year-old woman with dysphagia, skin thickening restricted to her fingers and face, and episodic digital vasospasm in the cold.Anti-centromere antibodies
  • A 50-year-old man with progressive symmetric proximal muscle weakness, interstitial lung disease, and violaceous papules over his knuckles.Anti-Jo-1 (anti-histidyl-tRNA synthetase) antibodies
  • A 35-year-old woman with severe bilateral keratoconjunctivitis sicca, xerostomia, and an increased risk of B-cell lymphoma.Anti-Ro/SSA and anti-La/SSB antibodies

Answer

The clinical syndromes correctly map to the following markers: SLE with anti-dsDNA, CREST syndrome with anti-centromere, dermatomyositis (antisynthetase syndrome) with anti-Jo-1, and Sjögren syndrome with anti-Ro/SSA and anti-La/SSB.
Each clinical vignette presents a classic constellation of symptoms for a distinct rheumatologic or autoimmune disease. Correctly diagnosing the underlying condition from the clinical presentation allows for the accurate identification of its most specific autoantibody, which is crucial for confirming the diagnosis, assessing patient prognosis, and guiding targeted management strategies.

Step-by-Step Solution

1
Analyze the first clinical vignette to identify Systemic Lupus Erythematosus (SLE) complicated by lupus nephritis.
Matched with its highly specific marker, anti-dsDNA.
Anti-dsDNA correlates with SLE disease activity, flares, and subsequent renal damage.
2
Identify the second vignette as limited cutaneous systemic sclerosis (CREST syndrome) based on the presence of Raynaud phenomenon, esophageal issues, and sclerodactyly.
Matched to anti-centromere antibodies.
Anti-centromere is the defining autoantibody for the limited cutaneous form of systemic sclerosis.
3
Recognize the third vignette as dermatomyositis with associated interstitial lung disease, characteristic of antisynthetase syndrome.
Matched to anti-Jo-1 antibodies.
Anti-Jo-1 (an anti-tRNA synthetase) is highly specific for inflammatory myopathies that present with interstitial lung disease and mechanic's hands.
4
Diagnose the fourth vignette as Sjögren syndrome based on the prominent sicca symptoms and the well-documented heightened risk for B-cell lymphoma.
Matched to anti-Ro/SSA and anti-La/SSB antibodies.
These specific ribonucleoprotein antibodies are the hallmark serological findings required to formalize a Sjögren syndrome diagnosis.

Key Concept

Identifying specific autoantibodies for rheumatologic and autoimmune diseases based on clinical manifestations is essential for establishing an accurate diagnosis, anticipating organ involvement, and predicting disease complications.
Rate this question

Topics

Immunologic and Inflammatory Processes

More questions from this topic