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.
This is a preview of subscription content, access via your institution
Subscribe to Journal
Get full journal access for 1 year
only $6.58 per issue
All prices are NET prices.
VAT will be added later in the checkout.
Tax calculation will be finalised during checkout.
Get time limited or full article access on ReadCube.
All prices are NET prices.
Bird HA et al. (1979) An evaluation of criteria for polymyalgia rheumatica. Ann Rheum Dis 38: 434–439
Jones JG and Hazelman B (1981) The prognosis and management of polymyalgia rheumatica. Ann Rheum Dis 40: 1–5
Chuang TY et al. (1982) Polymyalgia rheumatica: a 10-year epidemiologic and clinical study. Ann Intern Med 97: 672–680
Naschitz JE et al. (1999) Rheumatic syndromes: clues to occult neoplasia. Semin Arthritis Rheum 29: 43–55
Schattner A et al. (1985) Temporal lobe meningioma masked by polymyalgia rheumatica. Isr J Med Sci 21: 441–444
Papaioannou CC et al. (1979) Case report: association of giant cell arteritis and pituitary tumor. Report of two cases. Am J Med Sci 277: 85–90
Warrington KJ et al. (2003) Acute myeloid leukemia associated with necrotizing temporal arteritis. J Rheumatol 30: 846–848
Espinosa G et al. (2002) Myelodysplastic and myeloproliferative syndromes associated with giant cell arteritis and polymyalgia rheumatica: a coincidental coexistence or a causal relationship? Clin Rheumatol 21: 309–313
Pankhurst T et al. (2004) Malignancy is increased in ANCA-associated vasculitis. Rheumatology 43: 1532–1535
Little MA et al. (2004) Polymyalgia rheumatica preceding small-vessel vasculitis: changed spots or misdiagnosis? QJM 97: 289–292
Guillevin L et al. (1997) A prospective, multicenter, randomized trial comparing steroids and pulse cyclophosphamide versus steroids and oral cyclophosphamide in the treatment of generalized Wegener's granulomatosis. Arthritis Rheum 40: 2187–2198
Scott DG et al. BSR & BHPR Guidelines for the Management of adults with ANCA associated vasculitis. J Rheumatol, in press
Tervaert JW et al. (2001) Novel therapies for anti-neutrophil cytoplasmic antibody-associated vasculitis. Curr Opin Nephrol Hypertens 10: 211–217
The authors declare no competing financial interests.
About this article
Cite this article
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). https://doi.org/10.1038/ncprheum0352