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[An autopsy case of malignant rheumatoid arthritis (MRA) which was difficult to distinguish from polyarteritis nodosa
T Hidaka1, K Suzuki, M Kawakami
1Second Department of Pathology, National Defense Medical College, Saitama.
Abstract:
We encountered a patient who was diagnosed as rheumatoid arthritis (RA) at 15 years old and developed malignant RA (MRA) within one year. He suffered from mononeuritis multiplex and cutaneous infarction. Despite of treatment including steroid pulse therapy, neuritis progressed. Lung infiltration, pancreatitis and intestinal bleeding were accompanied. He died of disseminated intravascular coagulation on 153 days after admission. Autopsy revealed systemic rheumatoid vasculitis in coronary artery, pancreas, liver, small and large intestine, kidney and lung. These severe vasculitis occurred in young RA patient are rare case and it is important to consider the therapy and prognosis.
Insights
A rare case of malignant rheumatoid arthritis (RA) rapidly developed in a 15-year-old, leading to severe systemic vasculitis and death. This highlights the aggressive potential of RA in young patients and the need for careful monitoring.
Area of Science:
- Rheumatology
- Pathology
- Clinical Medicine
Background:
- Rheumatoid arthritis (RA) is a chronic autoimmune disease primarily affecting joints.
- Malignant RA (MRA) is a rare, severe variant with systemic manifestations.
- Early-onset RA presents unique challenges in diagnosis and management.
Observation:
- A 15-year-old patient diagnosed with RA rapidly progressed to MRA within a year.
- The patient experienced severe complications including mononeuritis multiplex, cutaneous infarction, lung infiltration, pancreatitis, and intestinal bleeding.
- Despite aggressive treatment with steroid pulse therapy, neurological complications worsened.
Findings:
- Autopsy confirmed systemic rheumatoid vasculitis affecting multiple organs: coronary artery, pancreas, liver, intestines, kidneys, and lungs.
- Disseminated intravascular coagulation (DIC) was identified as the cause of death.
- The case demonstrates an unusually aggressive and widespread vasculitic process in a young RA patient.
Implications:
- Severe systemic vasculitis in juvenile RA is a rare but critical clinical concern.
- Prompt recognition and aggressive management strategies are crucial for improving outcomes in MRA.
- This case underscores the importance of considering MRA in young RA patients presenting with severe systemic symptoms.