Jornal de Vasculite

Since that time, huge discussion has existed with regards to whether Sneddon condition is an unmistakable problem, part of a range of problems, or a subtype of antiphospholipid disorder. A few specialists accept that Sneddon disorder ought to be isolated into essential and optional cases. Essential Sneddon condition would signify situations where there was no known reason auxiliary Sneddon disorder would mean cases that are accepted to happen optional to another issue or thrombophilic state. A few specialists accept that Sneddon disorder ought to be separated by whether antiphospholipid antibodies are available or missing others examines immune system provocative etiopathogenesis versus thrombophilia.

Granulomatosis with Polyangiitis (GPA) is an anti-neutrophil cytoplasm antibody (ANCA) associated vasculitis that rarely presents as a solitary renal mass. We present a 57-year-old male who presented with abdominal pain and was found to have a complex right renal lesion on abdominal imaging. He later developed fevers, hypoxic respiratory failure and acute kidney injury. Chest imaging showed confluent consolidative changes in the mid to lower lung zones. He was started on antibiotics but continued to worsen. He was noted to have diffuse alveolar hemorrhage on bronchoscopy with autoantibodies to proteinase-3 (PR-3) returning positive. He was started on rituximab and steroids with dramatic improvement. Kidney biopsy revealed crescentic and necrotizing glomerulonephritis associated with ANCA-associated vasculitis. GPA presenting as a renal mass presents a diagnostic challenge. Prompt diagnosis is however paramount given how fast GPA can progress.

IgA vasculitis is poorly understood in adults and there is a greater need for randomized control trials to identify the clinical course and proper management of the disease. This is especially critical for adults with IgA vasculitis, given the association with worse outcomes and greater morbidity and mortality related to the disease’s comorbidities. For this reason, prompt identification and diagnosis of the vasculitis is critical. Importantly, cutaneous eruption is the most common presenting sign of IgA vasculitis, and it may be the only feature available to determine initial suspicion for IgA vasculitis. Atypical cutaneous features, such as necrotic or hemorrhagic purpura, bullae, or pustules, are more common in the cutaneous eruption of adult IgA vasculitis. This complicates identifying an already rare vasculitis in adults and could lead to delays in identification and management of the associated comorbidities. In this retrospective case series, we studied 34 adult patients with biopsy proven IgA vasculitis and divided each case into one of three different groups based on the dermatology providers’ differential diagnosis and initial clinical suspicion for IgA vasculitis at the time of biopsy. We then evaluated whether the proportion of patients with atypical cutaneous features differed across the groups. Atypical cutaneous features were significantly more common in cases where IgA vasculitis was not included in the differential compared to cases where IgA vasculitis was included. This suggests that atypical cutaneous features could be deterring clinicians from considering and identifying the vasculitis. This has implications for patient care related to the disease’s comorbidities and steps should be taken to emphasize that atypical cutaneous features are a common occurrence in adult IgA vasculitis.

ANCA test has diagnostic significance on AAV. The Immunofluorescence Assay (IFA) usually has a perinuclear staining pattern (PANCA), and Enzyme-Linked Immuno Sorbent Assays (ELISA) detect antibodies to Myeloperoxidase (MPO-ANCA) in MPA. Kidney involvement is noted in approximately 80-100 percent of patients with MPA cases, and the clinic spectrum range from microscopic hematuria to severe kidney injury with dialysis requirement. Pulmonary involvement is noted in approximately 25-55 percent of patients with MPA. Several observations and presumptions have been noted about TIN accompanying crescentic glomerulonephritis. This diversity may be considered as only a simple histological elaboration. However, it is a significant clinical entity for guiding the treatment.