Anti-neutrophil cytoplasmic antibody-associated vasculitis presenting with acute pancreatitis, jaundice, severe anaemia and upper gastrointestinal bleed.
Reddy S, Dhooria A, et al. • BMJ case reports • 2026
A young man with recurrent ocular and oral symptoms presented with moderately severe acute pancreatitis, upper GI bleeding, severe anaemia, and jaundice and was diagnosed with probable anti-neutrophil cytoplasmic antibody-associated vasculitis, responding well to immunosuppressive therapy with resolution of acute pancreatitis and GI bleed.
Key Findings
Background
AAV can present predominantly with gastrointestinal symptoms including acute pancreatitis, upper GI bleeding, jaundice, and severe anaemia in the absence of overt respiratory or renal involvement.
The patient was a young man who presented in the second week of moderately severe acute pancreatitis
Presentation included upper GI bleeding, jaundice, and severe anaemia as predominant features
No overt respiratory or renal involvement was documented at presentation
The case is described as a rare presentation of AAV
Background
The patient had a history of recurrent ocular and oral symptoms prior to his acute gastrointestinal presentation.
Recurrent ocular symptoms were noted in the patient's history
Recurrent oral symptoms were also part of the prior clinical history
These preceding symptoms may represent early or extra-gastrointestinal manifestations of AAV
The prior symptoms were not initially linked to a vasculitic process
Results
Diagnosis of probable AAV was established based on serology and biopsy findings after excluding alternative diagnoses.
ANCA serology was positive and contributed to the diagnosis
Biopsy findings were consistent with AAV
Alternative diagnoses were actively excluded before arriving at the diagnosis of probable AAV
The diagnosis was classified as 'probable' AAV
Results
The patient responded well to immunosuppressive therapy with resolution of acute pancreatitis and gastrointestinal bleeding.
Immunosuppressive therapy was initiated following diagnosis of probable AAV
Resolution of acute pancreatitis was achieved with treatment
Upper GI bleeding resolved following immunosuppressive therapy
The favorable response supports the vasculitic etiology of the GI manifestations
Conclusions
A high index of suspicion for vasculitis is necessary in cases of unexplained acute pancreatitis and/or GI bleeding, with active pursuit of serology and biopsy to improve outcomes.
The authors highlight that AAV presenting with predominant GI symptoms is rare
Serology and biopsy are recommended as diagnostic tools when vasculitis is suspected
Early diagnosis and treatment with immunosuppression was associated with favorable outcomes in this case
The case underscores the importance of considering vasculitis even when classic respiratory or renal features are absent
What This Means
This research presents a case report of a young man who developed a severe combination of symptoms — inflammation of the pancreas (acute pancreatitis), bleeding in the upper digestive tract, yellowing of the skin (jaundice), and dangerously low red blood cell counts — which were eventually traced back to a rare immune system disorder called ANCA-associated vasculitis (AAV). AAV is a condition where the body's immune system mistakenly attacks small blood vessels, and it is most commonly recognized when it affects the kidneys or lungs. In this patient, however, the digestive system was the primary target, making the diagnosis much harder to identify.
The diagnosis was only reached after doctors performed blood tests looking for specific antibodies (ANCA serology) and obtained tissue samples (biopsy), and after ruling out other possible causes. Notably, the patient had also experienced recurring eye and mouth problems in the past, which in retrospect may have been early signs of the same underlying condition. Once treated with medications that suppress the immune system, his pancreatitis and GI bleeding resolved, suggesting that the vasculitis was directly causing these problems.
This research suggests that doctors should consider vasculitis as a possible cause when patients present with unexplained pancreatitis or gastrointestinal bleeding, even when the more typical signs of vasculitis — such as kidney or lung involvement — are absent. Performing ANCA blood tests and tissue biopsies early in such cases could help reach the correct diagnosis faster and lead to more effective treatment, potentially preventing serious complications.
Reddy S, Dhooria A, Kansal D, Chidambaram M, Gupta O, Balankhe K, et al.. (2026). Anti-neutrophil cytoplasmic antibody-associated vasculitis presenting with acute pancreatitis, jaundice, severe anaemia and upper gastrointestinal bleed.. BMJ case reports. https://doi.org/10.1136/bcr-2026-274658