Consider this: There’s more to asthma if you think outside the box
MDlinx Oct 11, 2024
As the last day of CHEST winds down, an afternoon session, Insights Into Asthma Plus Entities, explored eosinophilic lung diseases. By looking at a few case-based discussions, the hosts emphasized competency in identifying and treating multiple conditions.
“There are many disorders in the group of eosinophilic lung diseases. Some are idiopathic, and some have unknown etiologies,” Stephanie Levine, MD, FCCP said. “Many overlap with asthma, and most are corticosteroid-response.”
Dr. Levine then kicked off the discussion with a case: “A 50 yo woman presents with 2 months of low-grade fevers, cough, a 5 lb. weight loss and progressive SOB. She has a 15-year h/o asthma and only uses prn albuterol. Labs are notable for 45% eosinophils in blood. IgE is 400 IU/L. ANCA is negative. PFTs reveal.”
She asked the crowd what the likely diagnosis could be. Choosing from four options (chronic eosinophilic pneumonia, allergic bronchopulmonary aspergillosis, eosinophilic granulomatosis with polyangiitis, and hypereosinophilic syndrome), the room dialed in their votes. The vast majority (88%) chose correctly: chronic eosinophilic pneumonia (CEP).
Consider this: There’s more to asthma if you think outside the box
Voices from the floor
Dr. Levine explains that CEP is rare, idiopathic, more common in women, common in people 30-50 years of age, and occurs in non-smoker. To diagnose, clinicians should look at clinical present, imaging, bronchoscopy (to exclude infection), exclude other etiologies like travel.
“Treatment involves corticosteroids for three to six months,” she adds. “In severe or progressive, use IV corticosteroids.” Generally patients see improvement over the next few days, often within 48 hours.
Alternative options may include inhaled corticosteroids, and certain biologics, like mepolizumab and benralizumab, according to case data the session hosts shared.
Hypereosinophilic syndromes (HES)
Next up, Malvika Kaul, MD, discussed hypereosinophilic syndromes (HES), presenting another case and poll for the room: “A 45-year-old man with previously well-controlled asthma on ICS/LABA presents with worsening dyspnea, requiring numerous prednisone bursts and ER visits over the past 6 months. He had an elevated troponin but normal EKG. Additional work up is notable for peripheral eosinophilia on multiple occasions (AEC 1800-3000 cells/uL) and a negative strongyloides IgG. A chest CT showed a left upper lobe mass consistent with lung adenocarcinoma.”
The room was polled on the best next step for this patient, and most (95%) of the room agreed that the patient needed to have a bronchoalveolar lavage performed. This time, the room as a whole was wrong.
“The answer is C,” Dr. Kaul said. “The answer is blood molecular testing for FIP1L1-PDGFRA mutations in blood.” Only 5% of participants selected this option in the poll.
Voices from the floor
HES, Dr. Kaul explained, is an absolute eosinophil count of more than 1,500 cells per uL. “The more eosinophils the more it narrows the diagnosis,” she says.
To diagnose, she says, “You need a good health history and a physical exam,” looking at things like travel history, sinus and nasal disease, neuropathy, and skin issues. “This is a disorder that causes systemic problems, especially with skin or cardio.”
You’ll also want to do a diagnostic workup including, CBC diff, liver and kidney function, troponin, parasite serology, PFT, CT imaging, and biopsy.” More so, you’ll want to follow up for initial abnormal findings with colonoscopy, MRI, allergy/immunology, and hematology.
“Think about categorizing HES in two ways—low risk and high risk,” Dr. Kaul says. High risk includes HE with hypereosinophilia with end organ dysfunction and severe complications. These patients should be given high-dose corticosteroids and/aor other therapies while continuing workup. “You want to start treatment really quickly,” she says, especially in high risk cases.
Direct takeaway
There are many HES subcategories, too, and the most common is the overlap, which is single organ or syndromic HES. Others include myeloid, lymphocytic, familiar, HEUS, associated, and idiopathic. “It is important to recognize myeloid, because this treatment is very different,” Dr. Kaul clarifies—and pulmonologists may not even treat this.
For HES and it subtypes—excluding myeloid HES—anti-IL5 therapies are effective and safe. The session hosts referred to the Journal of Allergy and Clinical Immunology’s findings: “Low dose mepolizumab is a safe and effective steroid sparing option in [idiopathic] HES remission maintenance. It might represent a further step towards a precision medicine approach to a still challenging immunological disease.”
Eosinophilic granulomatosis with polyangiitis (EGPA)
Next, Syed Shahzad Mustafa, MD, discussed eosinophilic granulomatosis with polyangiitis (EGPA), for which there is no real diagnostic criteria and based on expert opinion. To diagnose it, eosinophil count should be over 1000 cells/microL. You’ll want ANCA testing, IgE levels, and biopsy of tissue showing eosinophilic infiltration in vessel walls, vasculitis, or necrotizing granulomatous disease.
“Be aware of the overlap in presentation with other vasculitis syndromes…and other disease processes,” he says.
Treatment is “unfortunately” corticosteroids, Dr. Mustafa says, although DMARDS, mepolizumab, and benralizumab are also options, as are B-cell depletion and cyclophosphamide.
When it comes to EGPA, “working with a multidisciplinary team is important in optimizing patient care,” he adds.
A note on steroid stewardship
He ended by reminding the room about steroid stewardship—a topic that came up a lot across sessions at this year’s CHEST.
“Really think about steroid-sparing agents,” he stressed. “We want to get away from systemic steroids,” he says. “We know that 1000 mg of steroids cumulatively in life has a meaningful impact in life.”
Biologics like omalizumab and dupilumab, Dr. Mustafa adds, offer an effective, safe option.
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