Current clinical and therapeutic approach to tumour-like mass lesions in granulomatosis with polyangiitis.

Granulomatosis with polyangiitis (GPA) is a systemic autoimmune disorder classified among the anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and characterized by a triad of upper and lower respiratory tract disease, systemic vasculitis involving small-to-medium vessels and renal manifestations. Mass lesions, also described as inflammatory lesions, pseudotumor or tumour-like masses, are uncommon manifestations of GPA and are often called granuloma since histology examination shows granulomatous inflammation and rarely vasculitis. Masses could represent a localized manifestation of GPA or develop as part of a systemic disease. Unusual clinical presentation together with nonspecific radiological and histological features may delay the correct diagnosis leading to disease progression and organ damage. Diagnosis of GPA in such cases may be challenging and malignancy or infections must be considered as alternative diagnostic options. Here we reviewed all the different sites where mass lesions were reported in GPA, focusing on atypical localization, and summarized current therapeutic options and their different outcomes. We retrieved and discussed the cases reported since 2010, bearing in mind the advances in the therapeutic management of AAV patients in the last decade, namely biological therapy such as rituximab. Despite treatment regimens with glucocorticoids and immunosuppressive agents, mass lesions have a refractory course in a high proportion of patients. Invasive surgical procedures may be considered only when drug therapy fails.

Biologic therapy in relapsing polychondritis: navigating between options.

INTRODUCTION: Relapsing polychondritis (RP) is a rare systemic inflammatory disease of unknown etiology, primarily affecting cartilaginous tissue and proteoglycan-rich structures. Clinical manifestations vary from mild symptoms to occasional organ or life-threatening complications. Treatment can be challenging and is mostly based on experience or case reports/series. AREAS COVERED: There is growing literature investigating the role of biologics in the management of RP. TNFα antagonists, abatacept, tocilizumab, rituximab, anakinra and tofacitinib have been prescribed in several RP patients, mainly as second-line treatment, after conventional immunosuppressive agents' failure. EXPERT OPINION: Glucocorticoids represent the gold standard treatment of RP. Conventional immunosuppressants should be administered in refractory patients or when a glucocorticoid-sparing effect is needed. Biologic therapy should be used after failure of conventional treatments or in severe manifestations. TNFα inhibitors are the most prescribed biologic agent, with partial or complete response in several cases, but loss of efficacy may occur over time. Infliximab and adalimumab should be preferred among TNFα antagonists. Abatacept and tocilizumab proved to be effective as second-line biologic agents, but infections are reported with the former. Data on anakinra and rituximab are controversial, therefore they are not recommended as first-line biologic drugs. The use of JAK inhibitors is still anecdotal.