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We present a case of a man in his 40s with pulmonary-renal syndrome due to myeloperoxidase-positive antineutrophil cytoplasmic antibodies-associated vasculitis and concurrent cold agglutinin disease, a combination that has not yet been described in the literature. The fulminant course of the disease, including the need for kidney replacement therapy and mechanical ventilation posed a significant treatment challenge due to haemolytic complications., Competing Interests: Competing interests: JL received consulting/lecture fees from Vifor Pharma., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Drug-induced vasculitis can rarely cause inflammation and necrosis of blood vessel walls of both kidney and lung tissue. Diagnosis is challenging because of the lack of difference between systemic and drug-induced vasculitis in clinical presentation, immunological workup and pathological findings. Tissue biopsy guides diagnosis and treatment. Pathological findings must be correlated with clinical information to arrive at a presumed diagnosis of drug-induced vasculitis. We present a patient with hydralazine-induced antineutrophil cytoplasmic antibodies-positive vasculitis with a pulmonary-renal syndrome manifesting as pauci-immune glomerulonephritis and alveolar haemorrhage., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

A previously healthy man in his 20s presented with acute respiratory distress syndrome and subconjunctival haemorrhage. Imaging was indicative of pervasive pulmonary haemorrhage. There was no evidence of renal involvement. The patient rapidly deteriorated with aggravating respiratory failure regardless of invasive mechanical ventilation and required extracorporeal membrane oxygenation (ECMO). This maintained the patient adequate time to allow aggressive therapy. Skin biopsy indicated leucocytoclastic vasculitis. Given that the patient was C-antinuclear cytoplasmic autoantibody (ANCA) positive, pulse dose steroids and rituximab were initiated for the suspicion of ANCA-associated vasculitis (AAV) which resulted in improvement of airspace disease and subconjunctival haemorrhage. Only a few cases reported successful use of ECMO in severe diffuse alveolar haemorrhage (DAH) due to AAV, but no case was in DAH combined with subconjunctival haemorrhage. The need for systemic anticoagulation with pre-existing haemorrhage is still a challenging dilemma., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

We present a patient with systemic symptoms including 4 months of dyspnoea worsened with exertion, fatigue, rhinorrhoea, intermittent facial swelling, generalised lymphadenopathy and weight loss. Laboratory studies demonstrated proteinuria and eosinophilia. His serology was consistent with Epstein-Barr Virus (EBV) reactivation. A lymph node biopsy was consistent with EBV-associated reactive lymphoid hyperplasia. He was told to continue symptomatic treatment for EBV infection. After several admissions, vasculitis workup and myeloperoxidase-antineutrophil cytoplasmic autoantibody (ANCA) studies were positive. Evolution of clinical symptoms, laboratory parameters and our literature review suggested the diagnosis of EBV-associated ANCA vasculitis. Steroids were started after the patient continued to deteriorate; the viral load started increasing, so we added valganciclovir with favourable clinical response and no relapse during the follow-up for 6 months. This suggests that with evidence of viraemia (primary or reactivation), antiviral treatment likely has clinical benefit while immunosuppression is being considered., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2021. No commercial re-use. See rights and permissions. Published by BMJ.)

We present a case of antineutrophil cytoplasmic antibodies (ANCA)-associated rapidly progressive glomerulonephritis in the context of treatment of pulmonary tuberculosis (TB). A 42-year-old woman was treated for drug-susceptible pulmonary TB and represented with paradoxical worsening of symptoms and radiological features. She was HIV negative. A severe acute kidney injury with features of glomerulonephritis was evident on admission. Perinuclear ANCA and antimyeloperoxidase antibodies were present in serum and renal biopsy was consistent with ANCA-associated vasculitis. The patient was successfully treated with both antituberculous therapy and immunosuppression (corticosteroids and mycophenolate mofetil) with subsequent clinical improvement and amelioration of renal function. We propose this is the first case that describes the association between paradoxical reactions during TB treatment and ANCA-associated glomerulonephritis., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2021. No commercial re-use. See rights and permissions. Published by BMJ.)

A 64-year-old man with a 2-year history of palindromic rheumatoid arthritis, presented with recurrent flares of arthritis, weight loss, new onset Raynaud's phenomenon and one previous episode of small-volume haemoptysis. Investigations, including renal biopsy, revealed antineutrophil cytoplasmic antibodies-mediated vasculitis. This case highlights the need to consider vasculitis in patients in whom there is an atypical history of arthritis., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2021. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Antineutrophil cytoplasmic antibodies associated vasculitis (AAV) presenting with muscle weakness is rarely reported. We report a case of myeloperoxidase positive vasculitis presenting with severe proximal muscle weakness with normal creatine kinase and no positron-emission tomography uptake. There was a significant delay in the diagnosis of AAV due to atypical presentation. We propose AAV be considered in the differential diagnosis of proximal muscle weakness after excluding the common causes., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2020. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Systemic sclerosis (SSc) is characterised by non-inflammatory vasculopathy, autoimmunity and widespread fibrosis. While the presence of antineutrophil cytoplasmic antibodies (ANCAs) has been reported in SSc, their association with ANCA-associated vasculitis is exceedingly rare. Myeloperoxidase ANCA is more common than proteinase-3 ANCA, and glomerulonephritis is the most common clinical presentation of ANCA-associated vasculitis in SSc. ANCAs have been associated with the adverse disease outcomes in SSc, including higher mortality per recent reports. A 65-year-old man with diffuse cutaneous SSc for 6 years presented with new-onset peripheral neuropathy. Workup revealed a positive proteinase-3 and cytoplasmic ANCA, and histopathology confirmed an inflammatory vasculitic neuropathy. The patient was successfully treated with rituximab. Our case highlights the importance of checking ANCA in SSc at baseline, given the risk of disease-related complications, even years later. Tissue biopsy is often warranted for confirmation of vasculitis and prompt treatment can optimise long-term outcomes., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

A 77-year-old man was admitted with a relapse of antineutrophil cytoplasmic antibody-positive vasculitis with pulmonary involvement and acute kidney injury. There was a background of pulmonary fibrosis (non-specific interstitial pneumonia type pattern) and superadded pulmonary haemorrhage, acute pulmonary oedema and sepsis. The patient was intubated for 4 days and remained dependent on high flow oxygen and continuous positive airway pressure after extubation. A chest radiograph performed 2 weeks after extubation demonstrated unexpected, extensive pneumomediastinum and subcutaneous emphysema. This was confirmed on CT which raised the possibility of a tracheal defect at the level of the prior endotracheal tube cuff position. Tracheal injury was considered clinically unlikely due to the considerable interval since extubation and a short, uneventful intubation period. The cardiothoracic team recommended a diagnostic bronchoscopy but this was felt too high risk by the clinical team. The cause of pneumomediastinum and subcutaneous emphysema remained indeterminate., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Abnormalities in alpha-1 antitrypsin (AAT) proteins are risk factors for human disease. While the most common is AAT deficiency, a genetic disorder associated with chronic obstructive pulmonary disease, additional disorders associated with AAT abnormalities are increasingly recognised. We describe a middle-aged woman who presented with fulminant hepatic and multiorgan failure. Evaluation revealed the patient to have a rare AAT phenotype PiEE. Her clinical presentation was consistent with antineutrophilic cytoplasmic antibody-associated vasculitis, and her history suggested features of panniculitis. This is the first description of this rare homozygous AAT phenotype and possible disease associations with the 'E' protein. Given that abnormal AAT are under-recognised, and that new mutations and phenotypes continue to be identified, we will need to expand on our knowledge base and report clinical manifestations associated with these abnormal phenotypes., Competing Interests: Competing interests: RR is on Speaker’s Bureau for Grifols., (© BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Competing Interests: Competing interests: None declared.

Rapidly progressing glomerulonephritis like microscopic polyangiitis and allergic granulomatous angiitis are among the common presentations of perinuclear antineutrophilic cytoplasmic antibody (p-ANCA) vasculitis. Involvement of central nervous system is rare in contrast to mononeuritis multiplex, which is a well-known neurological manifestation of this condition. We report a case presented with uraemic encephalopathy and posterior reversible encephalopathy syndrome (PRES)-related symptoms, which showed recovery after haemodialysis although PRES with seizures recurred later. As uraemic encephalopathy appears to be the underlying aetiology as per the temporal correlation of correction of uraemia and resolution of the symptoms of PRES, it becomes a rare case of uraemia-induced PRES as a presenting manifestation of p-ANCA-associated vasculitis along with necrotising crescentic glomerulonephritis., (2014 BMJ Publishing Group Ltd.)

We report the case of a 51-year-old African woman with longstanding well-controlled HIV who developed relapsing systemic antineutrophil cytoplasm antibody (ANCA)-associated vasculitis and glomerulonephritis. She presented with an acute kidney injury and heavy proteinuria in the setting of a prolonged history of diffuse arthralgias and transient ocular symptoms. Antimyeloperoxidase (MPO) antibody titre was >100 IU/mL. Subsequent renal biopsy demonstrated a focal necrotising pauci-immune glomerulonephritis and a diagnosis of MPO-ANCA-associated microscopic polyangiitis was made. The patient was treated with tapering glucocorticoids and rituximab with resolution of her acute kidney injury and reduction in her proteinuria. Treatment was complicated by the development of steroid-induced diabetes and one mild clinical relapse, but was otherwise well tolerated without infectious complications or deterioration in her HIV disease.

We report a case of two patients with foot drop due to peroneal nerve infarct as early sign of two different forms of primary systemic vasculitides: a predominantly small-vessel p-antineutrophil cytoplasmic antibody-positive vasculitis (microscopic polyangiitis) and a predominantly medium-vessel vasculitis (polyarteritis nodosa).

The authors report the case of a 72-year-old man presenting with chronic dyspnoea and pyrexia of unknown origin (PUO). After extensive investigation, he was found to have pulmonary fibrosis with usual interstitial pneumonia pattern on high-resolution CT imaging and positive myeloperoxidase antineutrophil cytoplasmic antibodies (MPO-ANCA) with no infectious cause or other evidence of vasculitis organ involvement. His pyrexia and symptoms settled with oral systemic corticosteroid treatment. To the authors' knowledge this is the first case of MPO-ANCA positive vasculitis presenting as PUO and pulmonary fibrosis alone. The authors review the recent literature regarding the association of pulmonary fibrosis with ANCA-positive vasculitides with regards to pathogenesis, prognosis and treatment.