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Nursing Care and Considerations with Granulomatosis in the Emergency Department Ann Jimenez, BSN, RN, CCRN, CEN - Carilion Giles Community Hospital Emergency Department
Mrs.G presents to the emergency department with complaints of shortness of breath and worsening voice hoarseness. She was quickly assisted into her wheelchair while her spouse, who accompanied her, was questioned about her health history. He explained that she has Wegener’s Granulomatosis. Granulomatosis with polyangiitis (GPA, formerly known as Wegener’s Granulomatosis) is an autoimmune small-vessel vasculitis causing inflammation. It is a rare multisystem disease, often affecting the sinuses, lungs, and kidneys. It has no known etiology and requires several studies to confirm the diagnosis. Many factors play a role in diagnosing GPA. Symptoms that support a diagnosis include a runny nose, hemoptysis, dyspnea, and fatigue, all of which conspicuously do not improve (Lutalo, D’Cruz, 2014). Early diagnosis is essential in beginning treatment because GPA can worsen rapidly. A patient can be diagnosed by a common blood test called anti-neutrophil cytoplasmic antibody (ANCA), which identifies a specific antibody in serum. A positive ANCA test alone cannot confirm the diagnosis, so a tissue biopsy may still be needed. Once a GPA diagnosis is confirmed, treatment regimens will be dictated by the particular organ systems involved, as well as by medications that treat systemic inflammation (Falk, Merkel, King, 2019). Treatment regimens will include glucocorticoids and immunosuppressive medications. Nurses will be familiar with most of the medications that are used in the treatment of GPA because these medications are frequently seen on medication lists of patients with organ transplants, rheumatoid arthritis, lupus, and irritable bowel disease, in addition to other autoimmune diseases. Once GPA symptoms are controlled, patients may be required to take medications to prevent a relapse, like methotrexate and rituximab. The hallmark medication combination is prednisone and Cytoxan, which provide insight into whether remission can be achieved. However, there are side effects that must be considered, including hair loss, nausea, and diarrhea. In particular, severe immunosuppression will need to be taken seriously. Regular monitoring of complete blood count, renal function, liver function, urine studies, and clinical symptoms is recommended. Plasma exchange, known as plasmapheresis, may be considered in those patients where medication treatment has failed. Plasmapheresis works by eliminating ANCA from the body circulation in those patients with life-threatening renal vasculitis (Lutalo, D’Cruz, 2014). Initially, nursing care of a patient with GPA will always focus on airway, breathing, and circulation. This may sound elementary but should guide an emergency department nurse’s care. The nurse should place the patient on the heart monitor, blood pressure, and oxygen saturation monitors and establish IV access. Anticipated care includes oxygen, nebulization treatments, IV corticosteroids, and possibly anxiolytics since anxiety can potentiate symptoms. The physician and respiratory therapist should be called to the bedside promptly. Mrs. G was quite anxious and breathless with audible stridor, caused by GPA-induced subglottic stenosis and supraglottic inflammation. Joining the team at the bedside, the ED physician guided her care. While the nurses connected her to heart and vital sign monitors and started a saline lock, the respiratory therapist treated her with a racemic epinephrine nebulizer.
