What is the diagnosis for an adult with joint pain, synovitis of the arm, pleuritic chest pain, pulmonary embolism (PE), pancytopenia, elevated antinuclear antibody (ANA) titer, and significantly elevated ferritin level without a rash?

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Diagnosis: Systemic Lupus Erythematosus (SLE)

The most likely diagnosis is SLE (Option B), based on the positive ANA at 1:320, pancytopenia, pleuritic chest pain with pulmonary embolism, and joint involvement—all characteristic features of lupus, while the ferritin level of 900 ng/mL is too low and the absence of fever/rash argues strongly against Adult-Onset Still's Disease. 1

Key Diagnostic Reasoning

Why SLE is the Correct Answer

  • ANA 1:320 strongly supports SLE: This titer exceeds the diagnostic threshold and provides 95.8% sensitivity and 86.2% specificity for systemic autoimmune rheumatic diseases when combined with clinical features 1

  • Pancytopenia is characteristic of SLE: Cytopenias including anemia, leukopenia, and thrombocytopenia are hallmark features of lupus 1

  • Pleuritic chest pain with PE fits SLE: Serositis (pleuritis) is a classic SLE manifestation, and lupus patients have increased thrombotic risk due to antiphospholipid antibodies 1

  • Joint pain and synovitis occur in SLE: Arthralgia and non-erosive arthritis are common lupus manifestations 1

Why NOT Adult-Onset Still's Disease (AOSD)

  • Ferritin level is far too low: AOSD typically presents with ferritin >1000 ng/mL, often ranging from 4000-30,000 ng/mL, with extreme levels up to 250,000 ng/mL reported 2. The ferritin of 900 ng/mL argues strongly against AOSD 1

  • No characteristic rash: AOSD requires an evanescent salmon-pink rash that coincides with fever spikes, typically on the trunk—this patient has no rash 2

  • No fever pattern described: AOSD diagnosis requires spiking fever ≥39°C for at least 7 days 2. The absence of documented fever makes AOSD unlikely

  • ANA is typically negative in AOSD: While not absolute, ANA and rheumatoid factor are usually absent in AOSD 2

  • Pancytopenia in AOSD suggests MAS: When pancytopenia occurs in Still's disease, it should alert physicians to macrophage activation syndrome, a severe complication requiring prompt immunosuppressive treatment 2

Why NOT Rheumatoid Arthritis (RA)

  • Systemic features too prominent: RA does not typically present with pleuritic chest pain, PE, pancytopenia, and markedly elevated ferritin 1

  • ANA positivity at this titer is uncommon in RA: While low-titer ANA can occur in RA, 1:320 suggests a different systemic autoimmune disease 1

Why NOT Infective Endocarditis

  • Normal cardiac exam: The question explicitly states normal cardiac examination, making endocarditis unlikely 1

  • Clinical pattern fits autoimmune disease: The constellation of positive ANA, pancytopenia, and serositis points to autoimmune rather than infectious etiology 1

Essential Next Steps for Confirmation

Immediate Testing Required

  • Anti-dsDNA antibodies: Highly specific for SLE using Crithidia luciliae immunofluorescence test 1

  • Anti-Smith (Sm) antibodies: Highly specific for SLE with strong confirmatory power 1

  • Urinalysis with microscopy: Essential to evaluate for lupus nephritis (proteinuria, cellular casts) 1

  • Complete autoantibody panel: Including anti-Ro, anti-La, and antiphospholipid antibodies to assess for thrombotic risk explaining the PE 1

  • Complement levels (C3, C4): Typically low in active SLE 1

Critical Clinical Pitfall

Do not be misled by elevated ferritin alone into diagnosing AOSD. The ferritin level of 900 ng/mL, while elevated, falls far below the typical AOSD range and is more consistent with general inflammatory disease 2, 1. The absence of the pathognomonic fever pattern and salmon-pink rash, combined with positive ANA and pancytopenia, makes SLE the clear diagnosis 2, 1.

References

Guideline

Systemic Lupus Erythematosus Diagnosis and Management

Praxis Medical Insights: Practical Summaries of Clinical Guidelines, 2025

Guideline

Guideline Directed Topic Overview

Dr.Oracle Medical Advisory Board & Editors, 2025

Professional Medical Disclaimer

This information is intended for healthcare professionals. Any medical decision-making should rely on clinical judgment and independently verified information. The content provided herein does not replace professional discretion and should be considered supplementary to established clinical guidelines. Healthcare providers should verify all information against primary literature and current practice standards before application in patient care. Dr.Oracle assumes no liability for clinical decisions based on this content.

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