Still Going Strong
Connerton A, Cole R, Glynn E, Durcan L, O’Connell P.
Beaumont hospital, Beaumont, Dublin 9
We present the case of a 73-year-old female with no past medical history who complained of three months of subjective fevers and malaise. A presumptive diagnosis of polymyalgia rheumatica had been made in primary care, based on axial stiffness and elevated inflammatory markers, and steroid therapy was initiated. At doses of 40 mg prednisolone per day her symptoms improved somewhat but deteriorated dramatically with any attempt to wean. She continued to complain of sore throat, fevers, and widespread joint pain and was admitted to hospital for further investigation.
Labs on admission were notable for elevated WCC (20.64), CRP(262), ESR(12), and hyperferritinaemia (12,588) with normal fibrinogen and triglyceride levels. CT thorax abdomen and pelvis demonstrated supraclavicular adenopathy. Bone scan showed tracer uptake bilaterally in knees, wrists and elbows likely degenerative. Non-specific tracer uptake in the medial aspect of her left ankle Her bone marrow showed an increase in macrophage population which did not meet the criteria for macrophage activation/ secondary haemophagocytic lymphohistiocytosis .
She was ultimately diagnosed as adult onset Still’s disease fitting the Yamaguchi criteria, resistant to steroid taper and tocilizumab was initiated. She has improved significantly on tocilizumab, her inflammatory markers have settled and she is regaining mobility. (see table 1)
The diagnosis of adult onset stills disease is one of exclusion but is strongly suggested by extreme hyperferritinaemia in the absence of a malignant haematologic or severe infectious trigger. The importance of outruling macrophage activation is highlighted by this case and also the need to re-evalaute elderly patients with a label of PMR who are resistant to steroid taper.