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    Subjects/Pathology/Paraneoplastic Syndromes
    Paraneoplastic Syndromes
    medium
    microscope Pathology

    A 58-year-old male smoker presents with a 3-month history of progressive proximal muscle weakness, myalgia, and difficulty climbing stairs. Serum creatine kinase is elevated at 2400 U/L. Chest X-ray reveals a 4 cm left hilar mass. Anti-Jo-1 antibodies are positive. What is the most appropriate next step in management?

    A. Start corticosteroids and refer for urgent lung cancer staging and biopsy
    B. Perform muscle biopsy to confirm dermatomyositis before any further workup
    C. Start high-dose methotrexate and observe for spontaneous remission of lung findings
    D. Initiate immunosuppressive therapy with azathioprine alone and defer oncology evaluation

    Explanation

    Clinical Context

    This patient presents with dermatomyositis (DM) — evidenced by proximal muscle weakness, elevated CK, and anti-Jo-1 antibody positivity — in the setting of a lung mass on imaging.

    Key Pathophysiology

    Key Point
    Dermatomyositis is a paraneoplastic syndrome associated with underlying malignancy in 10–50% of adult cases, particularly lung cancer. The presence of a lung mass on imaging in a patient with newly diagnosed DM is a red flag for occult malignancy.

    Management Algorithm

    Loading diagram...

    Rationale for Correct Answer

    1. 1.
      Concurrent dual management: Corticosteroids address the inflammatory myositis, while urgent lung cancer staging and biopsy confirm malignancy and guide oncologic therapy.
    2. 2.
      Timing is critical: Paraneoplastic DM often improves with treatment of the underlying cancer; delaying cancer diagnosis worsens prognosis.
    3. 3.
      Anti-Jo-1 positivity is associated with interstitial lung disease and is a strong predictor of malignancy-associated myositis.

    High-Yield Facts

    High-YieldNEET PG
    In any adult presenting with dermatomyositis, particularly with anti-synthetase antibodies (Jo-1, PL-7, EJ), a thorough malignancy screen (CT chest/abdomen, age-appropriate cancer screening) is mandatory before attributing symptoms solely to autoimmunity.
    Clinical Pearl
    The presence of a lung mass on imaging in a DM patient is NOT incidental — it must be biopsied and staged urgently, as treatment of the underlying cancer often leads to remission of myositis.

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