ABSTRACT
Occupational exposure to silica has been seldom implicated to cause systemic sclerosis. Erasmus syndrome is a rare condition where there is development of systemic sclerosis following silicosis. Early diagnosis is essential for appropriate treatment and symptom control. We describe a 42-year-old stone cutter with silicosis who was subsequently diagnosed to have diffuse pattern of systemic sclerosis. A multidisciplinary team including pulmonologists, specialist palliative care physicians, rheumatologists and radiologists were involved in diagnosis and management. Timely diagnosis and a multidisciplinary team management with pharmacological and non-pharmacological measures was essential in holistic care provision. This is the first report of specialist palliative care team facilitating diagnosis of this rare syndrome and promoting integration in the overall care and management.
ABSTRACT
Takayasu arteritis (TA or TAK) is a chronic large vessel vasculitis with predilection to affect the aorta and its branches. The new 2022 ACR/EULAR classification criteria for Takayasu arteritis incorporated imaging characteristics as an absolute requirement. ESR and CRP fails in accuracy as disease activity markers. Pentraxin 3 appears to be a relatively superior biomarker, which correlates with ITAS 2010 as per several studies. PET-CT is also increasingly being studied for assessing disease activity with variable results. The management of TAK involves use of steroids with upfront steroid sparing immunosuppressive agents. MMF is one such conventional DMARD/immunosuppressant with good efficacy and better safety profile, as reported in various cohort studies. Tocilizumab is proved to be a rapid remission inducing agent in refractory Takayasu arteritis in observational studies. TNF inhibitors in many uncontrolled studies showed good responses, and there is a need for good RCTs for confirmation. JAK inhibitors have also been used with success in a few reports.
