An Unprecedented Triad: Simultaneous Acute Pancreatitis, Axonal Polyneuropathy, and Type 1 Diabetes Mellitus in a Catastrophic Juvenile Lupus Flare

TL;DR

A 14-year-old with lupus experienced a rare, severe flare involving pancreatitis, nerve damage, and diabetes, highlighting the need for vigilance in similar cases.

Contribution

This case reports the first documented simultaneous occurrence of acute pancreatitis, axonal polyneuropathy, and Type 1 Diabetes in juvenile lupus.

Findings

A hyperinflammatory lupus flare triggered widespread vasculitic injury and organ-specific autoimmunity.

Aggressive immunosuppression successfully treated the multi-system autoimmune crisis.

The case expands the known phenotypic spectrum of juvenile systemic lupus erythematosus.

Abstract

A 14‐year‐old female with juvenile systemic lupus erythematosus (jSLE) presented with a life‐threatening and previously unreported triad: the simultaneous onset of acute pancreatitis, severe axonal polyneuropathy, and autoimmune Type 1 Diabetes (T1D). This catastrophic, polysyndromic flare was driven by a hyperinflammatory state consistent with Macrophage Activation Syndrome, with the T1D diagnosis confirmed by high‐titer GADA, IA‐2A, and ZnT8A autoantibodies. This case provides a rare clinical model of a “perfect storm” in which a systemic inflammatory crisis appears to have inflicted widespread vasculitic injury while simultaneously acting as a “second hit” to trigger a latent, organ‐specific autoimmunity. Aggressive immunosuppression with pulse corticosteroids and mycophenolate mofetil led to remission of this multi‐fronted immunological attack. This case expands the phenotypic spectrum of jSLE to include a new paradigm of “lupus crisis,” demonstrating that a single flare can catastrophically shatter the boundaries between systemic and organ‐specific autoimmune diseases. It mandates a high index of suspicion for concurrent, multi‐lineage autoimmunity in patients presenting with severe, hyperinflammatory jSLE.

A severe juvenile systemic lupus erythematosus (jSLE) flare can manifest as a catastrophic crisis with the unprecedented simultaneous onset of acute pancreatitis, axonal polyneuropathy, and autoimmune Type 1 Diabetes. This case mandates a high index of suspicion for concurrent, life‐threatening complications in severely ill jSLE patients.

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