Innovative Treatments for Severe Raynaud’s and Multicentric Reticulohistiocytosis

The presentation reviewed difficult cases of severe Raynaud’s phenomenon and multicentric reticulohistiocytosis (MRH), highlighting newer treatment approaches. For Raynaud’s associated with systemic sclerosis, the speaker emphasized that severe disease can progress to digital ischemia, ulcers, scarring, and even auto-amputation, so evaluation should include a careful history for connective tissue disease. Standard management starts with warming measures and calcium channel blockers, with phosphodiesterase inhibitors such as sildenafil or tadalafil used more often now that cost barriers are lower. When these are insufficient, botulinum toxin injections can improve symptoms and tissue loss in many patients by reducing arteriolar contraction, and inpatient IV epoprostenol was described as a bridge therapy for threatened digits and severe ulcers, with retrospective data showing symptom improvement and some complete ulcer healing. The talk then shifted to MRH, a rare systemic disease marked by red papulonodules, coral-bead changes around nail folds, and often severe erosive arthritis; it can also be associated with malignancy, so cancer screening is important. In the presented patient, initial therapy with steroids, hydroxychloroquine, methotrexate, and infliximab was inadequate, but treatment with the JAK inhibitor upadacitinib led to near-complete skin clearance and major joint improvement, suggesting a promising option for refractory MRH.