RESUMO
We present a case of refractory cutaneous dermatomyositis (DM) in a 51-year-old Hispanic female which failed multiple treatments but found symptomatic relief with anifrolumab. Anifrolumab was the only treatment that was associated with significant improvement in the rash and pruritis of the patient and lowered her corticosteroid needs. To our knowledge, this is the only second case report that has shown success in treating refractory cutaneous symptoms of DM with anifrolumab after failing standard and multiple combinations of therapies. Anifrolumab is a new first-in-class human monoclonal antibody, which inhibits type 1 interferon receptor (IFN-1) and is used to treat systemic lupus erythematosus (SLE). It is FDA-approved for non-renal manifestations of SLE. This IFN pathway seems to be also active in patients with DM. The presence of IFN-1 and IFN-2 has been reported in muscle biopsies of patients with inflammatory myopathies. Moreover, the IFN activation signature is present in the muscle, blood, and skin of patients with DM. IFN-1 has been assumed to activate toll-like receptors which activate the dendritic cells leading to the secretion of cytokines and chemokines. This potential pathophysiological role of IFN in DM may explain the symptom improvement experienced by our patient after starting anifrolumab treatment. Anifrolumab has additionally been shown to have a good safety profile when used to treat patients with SLE with up to three years of treatment on background conventional disease-modifying antirheumatic drug (DMARD) therapies. In conclusion, SLE and DM share similarities in their pathophysiology and cutaneous disease involvement and can be differentiated clinically. Skin manifestations of DM can persist despite combinations of therapies even when weakness resolves. With this case report, we aim to highlight the possibility of utilizing anifrolumab for treating DM skin manifestations, especially in refractory cases. More research is needed to guide where anifrolumab stands in the therapeutic algorithm for DM. It is unknown whether it treats the myositis component, DM-related arthritis, or coexistent rheumatoid arthritis.
RESUMO
Myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) vasculitis manifests as a neutrophilic inflammation impacting small vessels across multiple organs, notably the lungs, kidneys, and skin. We present a unique case of MPO-ANCA vasculitis in a 77-year-old female characterized by glomerulosclerosis, rapidly progressive renal failure necessitating hemodialysis (HD), bullous skin lesions, and hypoxic respiratory failure. The patient, who had a history of type 2 diabetes, presented with progressive dyspnea, hypoxia, and acute kidney injury superimposed on chronic kidney disease (CKD) progressing to renal failure requiring dialysis. A renal biopsy highlighted globally sclerosed glomeruli, interstitial fibrosis, and tubular atrophy, along with increased immunoglobulin M (IgM) deposits on immunofluorescence, differing from typical findings. Prompt initiation of prednisone led to respiratory and cutaneous improvement; however, despite therapy, extensive renal damage led to the permanent requirement of dialysis. MPO vasculitis primarily targets small vessels, frequently affecting kidneys, with only a subset of patients progressing rapidly to end-stage renal failure necessitating HD, as observed in our case. Contrary to classical histopathological patterns, our patient exhibited augmented IgM deposits. Left untreated, MPO vasculitis with renal involvement poses a mortality risk of up to 90%, underscoring the significance of prompt detection and corticosteroid intervention to avert renal failure and improve patient outcomes. Early recognition and timely treatment are pivotal in mitigating the dire consequences of this condition, emphasizing the importance of considering MPO vasculitis in patients with rapidly deteriorating renal function.
