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A case of polymyalgia rheumatica, microscopic polyangiitis, and B-cell lymphoma


Background A 73-year-old, previously well woman was admitted to an emergency department because of a 3-month history of severe proximal girdle pain and stiffness with loss of appetite and weight. She was referred to a rheumatologist 10 days after her initial presentation. Within 4 weeks she presented to an outpatient clinic with nausea, vomiting, shortness of breath, painful mouth ulcers, rash on her legs and a further decline in appetite; she was readmitted to hospital. Within 4 months of initial presentation she became jaundiced.

Investigations At initial presentation, physical examination, biochemical, hematological and autoimmune screening, radiography of the pelvis, an abdominal ultrasound, and electromyography were conducted. At referral to a rheumatologist similar tests were repeated. At presentation to the outpatient clinic, hematological and biochemical screening, and a urine dipstick test were conducted. At readmittance to hospital, infectious and autoimmune screening, radiography of the chest, electrocardiogram, ultrasound of the abdomen, and renal biopsy were conducted. At the time of development of jaundice, biochemical and hematological screening, CT of the abdomen and ultrasound-guided biopsy of a pancreatic mass were conducted.

Diagnosis Polymyalgia rheumatica, antineutrophil-cytoplasmic-antibody-positive microscopic polyangiitis with renal involvement and B-cell lymphoma of the head of the pancreas.

Management The patient received oral prednisolone 15 mg daily for polymyalgia rheumatica along with alendronate 70 mg weekly. The patient received intravenous cyclophosphamide 500 mg and methylprednisolone 500 mg every 2 weeks for her microscopic polyangiitis with renal involvement. For B-cell lymphoma of the head of the pancreas, the patient received cyclophosphamide, doxorubicin, vincristine and prednisolone once monthly.

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Figure 1: Photographs from physical examination at the outpatient clinic
Figure 2: Photographs of renal biopsy
Figure 3: Histology of a specimen from the pancreatic head, showing a B-cell lymphoma with high malignant potential


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Correspondence to Kuntal Chakravarty.

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The authors declare no competing financial interests.

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Kuttikat, A., Keat, A., Hughes, R. et al. A case of polymyalgia rheumatica, microscopic polyangiitis, and B-cell lymphoma. Nat Rev Rheumatol 2, 686–690 (2006).

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