35. A referred case of probable PMR with a different outcome
Authors: Nawaz Asif , Rashid Yamin , Barber Julie 
Published: September 2019

Introduction

This is the case of a 67-year-old woman who presented to the rheumatology clinic with suspected diagnosis of polymyalgia rheumatica on prednisolone with complaints of flu-like illness, muscle aches and occasionally low-grade fever. Initial investigation showed anaemia with a significantly raised CRP and ESR. Further investigation revealed a different outcome.

Case description

A 67-year-old woman presented to the rheumatology clinic with the suspected diagnosis of polymyalgia rheumatica with prednisolone commenced in primary care. Three months before, she presented in primary care with a flu-like illness, muscle aches and occasionally low-grade fever. On investigation, she was found to have anaemia (Hb 92 gm/dl with MCV of 109 femtoliters per cell) with a significantly raised ESR of 123 mm/hr and CRP of 258 mg/L. Initially, there was a history of mild weight loss which she attributed to her diet and subsequently, she regained weight.

Her symptoms were not typical of PMR and upon further questioning, she complained of shortness of breath on inclines. She denied any symptoms of dizziness or any symptoms suggestive of embolic manifestation. Her previous medical history includes hypertension and hyperlipidemia for which she was on bendroflumethiazide and statins which were stopped in primary care three months back. Her blood pressure in the clinic was 156/77mmHg with a pulse of 82 beats per minute. There was no clubbing or any signs suggestive of polymyalgia rheumatica or any other connective tissue disease. On auscultation of heart, the first heart sound was loud and the chest was clear. Abdominal and neurological examination was unremarkable. Rest of investigations including urea and electrolytes, liver function test, ANA, anti ENA, Complement C3 &C4, immunoglobulins, creatine kinase, ferritin and thyroid function tests were unremarkable. An echocardiogram was requested as workup of unexplained raised inflammatory markers and non-resolving symptoms which showed a large echogenic mass in the left atrium attached to atrial septum and prolapsing through the mitral valve into the left ventricle, suggestive of left atrial myxoma. She was referred to cardiothoracic surgeons for resection. Her aches and pains subsided and she is doing very well after surgery. 

Discussion

Myxomas account for 40-50% of primary cardiac tumours. 75-85% occur in the left atrial cavity and up to 25% in the right atrium. Symptoms range from nonspecific and constitutional to sudden cardiac death. In 20% of cases, atrial myxoma may be asymptomatic and reported as an incidental finding. Other reported symptoms are exertional dyspnoea (75%) which may get worse leading to pulmonary oedema. Other symptoms may be due to obstruction of mitral valve leading to dizziness (20%), Symptoms of right heart failure, embolization to the central nervous system and retinal artery involvement may lead to blindness, coronary artery disease, and PE. Constitutional symptoms include fever, weight loss, arthralgias, and Raynaud’s phenomenon and are observed in 50% of patients, 15% of the atrial myxoma patients present with hemoptysis which may be due to an infraction or pulmonary oedema.

Laboratory studies include raised inflammatory markers including ESR and CRP, leukocytosis and anaemia. Transesophageal echocardiography has better specificity and 100% sensitivity compared to transthoracic echocardiography.

Histologic studies demonstrate the presence of lipidic cells embedded in a vascular myxoid stroma. Other investigation modalities include MRI, CT, and nuclear imaging.

Conventional treatment of atrial myxoma is surgical removal by median sternotomy. Mini thoracotomy with robotically assisted surgery has been reported, resulting in a shorter length of hospital stay, and it is considered a safe and feasible method for atrial myxoma excision. The investigators did not observe any difference in the quality of life between the two strategies. A case report of total endoscopic robotic resection of a left atrial myxoma in an elderly patient with persistent left superior vena cava also demonstrated the good outcome. No recurrence of myxoma was observed during the 2-year follow-up period if resected completely.

Key learning points

Polymyalgia rheumatica, raised inflammatory markers, malignancy, atrial myxoma, large vessel vasculitis.

Conflicts of interest

The authors have declared no conflicts of interest.