Correlate the following clinical patient profiles with their most likely underlying systemic rheumatologic diagnosis and associated extra-articular complication.
- A 58-year-old female with a 15-year history of symmetrical, erosive polyarthritis affecting the wrists and metacarpophalangeal joints presents with recurrent skin infections, an absolute neutrophil count of , and a palpable spleen.Rheumatoid arthritis complicated by Felty syndrome
- A 34-year-old male with a 10-year history of inflammatory back pain and bamboo spine on radiography presents with bounding peripheral pulses and a high-pitched, blowing diastolic murmur at the left sternal border.Ankylosing spondylitis complicated by aortic regurgitation
- A 29-year-old male with recurrent, painful oral and genital ulcers, as well as recurrent uveitis, presents with sudden-onset massive ascites, tender hepatomegaly, and jaundice.Behçet disease complicated by Budd-Chiari syndrome
- A 52-year-old female presenting with progressive symmetric proximal muscle weakness and a heliotrope rash now develops a dry cough, worsening exertional dyspnea, and bibasilar crackles.Dermatomyositis complicated by interstitial lung disease
- A 45-year-old female with severe Raynaud phenomenon, sclerodactyly, and dysphagia presents with a mean pulmonary arterial pressure of on right heart catheterization and a normal pulmonary capillary wedge pressure.Limited cutaneous systemic sclerosis complicated by pulmonary arterial hypertension
Cevap
The correct matches link each distinct clinical presentation to its classic extra-articular or systemic complication. The patient with erosive arthritis and neutropenia matches Rheumatoid arthritis with Felty syndrome. The patient with bamboo spine and a diastolic murmur matches Ankylosing spondylitis with aortic regurgitation. The patient with oro-genital ulcers and ascites matches Behçet disease with Budd-Chiari syndrome. The patient with a heliotrope rash and bibasilar crackles matches Dermatomyositis with interstitial lung disease. Finally, the patient with sclerodactyly and elevated pulmonary pressure matches Limited cutaneous systemic sclerosis with pulmonary arterial hypertension.
Each rheumatologic condition has highly specific systemic manifestations. Felty syndrome requires a background of RA; aortic root disease is a well-known cardiac complication of ankylosing spondylitis; Budd-Chiari syndrome reflects the hypercoagulable state of Behçet disease; interstitial lung disease is heavily linked to dermatomyositis (especially anti-Jo-1 positive); and pulmonary arterial hypertension is a classic complication of limited cutaneous systemic sclerosis.
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Anahtar Kavram
Identifying life-threatening extra-articular complications of common systemic rheumatologic diseases.