Rheumatoid arthritis associated vasculitis: a rare entity; case and review

Abstract

We present a case of a 60-year-old male with known seropositive rheumatoid arthritis and cerebral vasculitis who presented to the emergency room with abrupt onset lower back and abdominal pain. The patient developed peritonitis which led to an abdominal laparotomy where jejunal ischemia, necrosis, and perforation were found, requiring bowel resection. On pathology examination, the patient had mesenteric vessel intramural inflammation indicative of vasculitis. He developed an anastomotic leak on postoperative Day 4 and elected hospice care. A high index of suspicion for mesenteric vasculitis should be considered in patients presenting with abdominal pain in the setting of known rheumatoid arthritis associated vasculitis, especially patients with long-standing rheumatoid arthritis. The high mortality represented by gastrointestinal involvement in rheumatoid arthritis associated vasculitis warrants investigation in high-risk patients, despite its low prevalence. Treatment may consist of high-dose corticosteroids, immunosuppressive agents, biologic therapies that target the underlying autoimmune process, and in severe cases, bowel resection.

Introduction

Vasculitides is a term that describes several noninfectious inflammatory disorders of the vasculature [1]. The nonspecific clinical presentation of vasculitides with mesenteric involvement leading to intestinal ischemia poses several challenges, including timely diagnosis and referral to appropriate specialist care [2]. Vasculitides that can have bowel involvement include primary vasculitides, such as polyarteritis nodosa, IgA vasculitis, antineutrophilic cytoplasmic antibody (ANCA) vasculitis, Takayasu arteritis, Behçet’s; as well as secondary vasculitides, including systemic lupus erythematosus and rheumatoid arthritis (RA) associated vasculitis [2].

RA is one of the most common chronic autoimmune diseases characterized by systemic inflammation and damage to the synovial lining of joints. However, RA-related extra articular manifestations can have significant, unforeseen consequences. The development of necrotizing vasculitis of small or medium-sized vessels, referred to as RA associated vasculitis (RAAV), can affect various organ systems, leading to diverse clinical presentations. RAAV is a rare pathology that often affects patients with greater than a decade of known disease. The pathogenesis of RAAV is not quite fully understood and the inflammation is believed to result from an abnormal immune response although there are strong associations with certain genotypes of the disease [3, 4]. It occurs in ~1%–5% of patients with RA. While skin and peripheral nerve involvement are the most common manifestations of RAAV, GI involvement is found in <10% of patients resulting in small bowel or large bowel infarction, appendicitis, intrahepatic hemorrhage and pancreatitis. Definitive diagnosis is challenging as biopsy is required, typically obtained following resection.

In severe cases of RAAV with significant bowel involvement, bowel resection is necessary. Most often a finding of intestinal necrosis/perforation represents a grave prognosis with a terminal outcome. Alternatively, for cases with less severe bowel involvement, medical management is typically employed. This approach involves the use of medications and therapies to control inflammation, manage acute symptoms, and prevent disease progression. The specific treatment regimen may include corticosteroids, immunosuppressive agents and targeted biologic therapies to address the underlying immune responses. Modalities of case reports that were examined are illustrated in Table 1 for presentation and subsequent management/treatment of cases.

Case presentation

We present a case of a 60 year old man, who presented to the emergency room with abrupt onset lower back and abdominal pain. The patient had a known past medical history of seropositive RA with cerebral vasculitis, and prior cerebrovascular accidents. At presentation, the patient had been treated with one dose of Rituximab for maintenance therapy and stated he had missed his appointment 6 months prior for his injection.

Patient was evaluated with a CT scan of his abdomen with intravenous contrast and found to have some mesenteric inflammation with mild free fluid. At that time the patient was admitted for a septic work up. On patient’s hospital Day 2, his abdominal pain worsened and he was not responding to antibiotic treatment so was re-scanned and found to have concern for a perforated jejunal diverticulitis. On examination the patient was peritonitis and the decision was made to proceed to the operating room for exploration.

Upon entering the patient’s abdomen there was an area of middle jejunum with two necrotic perforations. Mesenteric venous and arterial thrombosis was noted, there was jejunal necrosis, edema up to the terminal ileum. At this time there arose concern for possible mesenteric ischemia and an intraoperative consult to vascular surgery was made.

Approximately 130 cm of jejunum was left, patient left in discontinuity and placed in a temporary abdominal closure. The following day, on return to the operating room patient found to have more necrotic jejunum, once resected a side-to-side stapled anastomosis was done, patient was closed and returned to the surgical intensive care unit for continued resuscitation.

During this time rheumatology was consulted for evaluation in the setting of patient’s RA and cerebral vasculitis for evaluation and recommendations. The patient’s pathology demonstrated diffuse acute and chronic vasculitis of the mesenteric vessels with thrombosis (Figs 1–3).

On postoperative day, four patients had worsening leukocytosis and abdominal pain, a computed tomography (CT) scan at that time demonstrated free fluid and inflammatory changes concerning for a possible anastomotic leak. Rheumatology weighed in that given the patient’s acute change and now-known pathology, treatment for mesenteric vasculitis was 3 days of 500 mg methylprednisolone.

The patient’s condition continued to worsen, goals of care discussion with the patient at that time was that another surgery would likely be an end-of-life event. The patient and family opted for hospice care and the patient died on hospital Day 18.

Discussion

Diagnosis of RA consists of a combination of clinical manifestations, rheumatoid factor (RF), anti-citrullinated protein antibodies (ACPA) and nonspecific inflammatory markers. RF seropositivity is an indicator of more extensive involvement, such as vasculitis, while high RF titers indicate poor prognosis [17].

Histopathology demonstrates mononuclear and neutrophilic infiltration and destruction of the vessel wall. Perivascular infiltrates in ≥3 cell layers of the vessel wall is a sensitive and specific finding that can differentiate between RAAV and non-RA vasculitis [3, 18]. About 1% of RA pts develop vasculitis, while 1%–10% of RAAV pts develop GI involvement [5].

Our review (Table 1) and experience with the case presented would indicate that physicians should have a high index of suspicion for mesenteric vasculitis in the patient presenting with abdominal pain in the setting of known seropositive RA.

Conclusion

A high index of suspicion for mesenteric vasculitis should be considered in patients presenting with abdominal pain in the setting of known RAAV, especially patients with long-standing RA. The high mortality represented by gastrointestinal involvement in RAAV warrants investigation in high-risk patients, despite its low prevalence. Considering RAAV in the differential diagnosis of patients with chronic RA and abdominal symptoms is critical. Early recognition and treatment of RAAV is imperative to ensure early intervention and coordination of specialist care. Treatment may consist of high-dose corticosteroids, immunosuppressive agents and biologic therapies that target the underlying autoimmune process and reduce inflammation.

Conflict of interest statement

None declared.

Funding

None declared.

References