Study Reports First Case of GPA Involving Entire Gastrointestinal Tract

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by Alice Melão |

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Gastrointestinal tract

Mexican researchers reported for the first time a patient with granulomatosis with polyangiitis (GPA) who experienced extensive, widespread gastrointestinal symptoms as the main manifestation of relapsing disease.

The case was reported in the study, “Extensive Gastrointestinal Manifestations as the Main Relapsing Disease in Granulomatosis with Polyangiitis,” published in the journal Digestive Diseases and Sciences.

The 43-year-old man was diagnosed with GPA at the age of 33. The disease initially caused fever and general malaise, with additional joint, ocular, and pulmonary symptoms. The patient also had autoantibodies against the proteinase 3 (PR3) protein.

He received treatment with glucocorticoids and methotrexate for six months, followed by methotrexate maintenance for the next five years, which ended in 2010.

Additionally, the patient had a history of hemorrhoidal disease, which was surgically treated in 2013 with complete resolution of the symptoms.

In 2016, he returned to the hospital with bloody stools and a high-grade fever after experiencing rapid weight loss for over a month. After an initial evaluation of his new symptoms, the patient received a second hemorrhoid surgery. However, he continued to experience persistent anal pain and bleeding with continuous fever, which was unsuccessfully medicated with antibiotics.

In addition to these symptoms, a physical examination revealed a hard mass in the anal region caused by the thickening of the rectal and anal walls. He also had arthritis affecting his knees and left wrist.

The clinical team performed upper and lower endoscopies to conduct a visual examination of the entire gastrointestinal (GI) tract. With this approach, doctors found large ulcers in several areas, including the pharynx, esophagus, duodenum, and colon.

Blood analysis showed markedly increased C-reactive protein and anti-PR3 antibodies, suggesting inflammation.

An X-ray evaluation of the chest revealed diffuse tissue infiltrates. But despite antibiotic treatment, the patient’s pulmonary symptoms progressed. Additional tests ruled out bacteria, fungal, or viral infections.

Tissue biopsies from several parts of the GI tract revealed small vessel inflammation, called vasculitis, with immune cell infiltrates throughout the tissues. This led to a final diagnosis of active GPA.

Doctors treated the patient with methylprednisolone, followed by prednisone and Rituxan (rituximab). After two years of follow-up, the patient remained asymptomatic, with all prior pulmonary and GI symptoms completely resolved. At this point, treatment with prednisone was discontinued and Rituxan was given as a maintenance therapy.

The course of his case is quite rare, as “GPA relapses usually follow the initial disease pattern,” the researchers wrote. “Indeed, less than 10% of relapsing patients have new organ involvement,” they added. Also, the disease rarely causes lower GI bleeding.

Despite the odd relapsing symptoms that affected the entire GI tract, all of the patient’s symptoms were adequately managed with glucocorticoids and Rituxan therapy, researchers observed.