Il dottor Marco Umberto Scaramozzino, Pneumologo, nello studio “La Madonnina” di Reggio Calabria si occupa di malattie dell’apparato respiratorio e condivide con la nostra redazione ed i lettori de “La voce dei medici” un case report (in lingua inglese) da lui realizzato.
The role of Mepolizumab in Overlap Syndrome (ACO): A strange case of ACO with cirrhosis of the liver secondary to primary cryptogenic cholangiopathy
Marco Umberto Scaramozzino1 – Aldo Diasparra2 Sapone Giovanni3 Giuliano Castignini4
1.MD U.O. Pulmonary Rehabilitation ICS Maugeri Marina di Ginosa (TA),
2. Acting Primary U.O. Pulmonary Rehabilitation ICS Maugeri Marina di Ginosa (TA),
3. Head of Nursing Department of Cardiology Polyclinic M.d.c. Reggio Calabria
4. Medical director Physiatrist U.O. Pulmonary Rehabilitation ICS Maugeri Marina di Ginosa (TA)
The purpose of this case report is to allow and approve the use of Mepolizumab in diseases that occur with hypereosinophilia and demonstrate its therapeutic role in patients with overlap syndrome (asthma + COPD – ACO). To date, there poor applications of use of mepolizumab in ACO, but there aren’t applications in literature of biologics in ACO + Cirrhosis of the liver secondary to cryptogenetic primary cholangiopathy reported in the literature.
Case report –A strange case of ACO with cirrhosis of the liver secondary to primary cryptogenic cholangiopathy
Former smoker patient, familiarity for bronchial asthma, recent diagnosis of liver cirrhosis secondary to primary cholangiopathy (histological examination of December 2020), in follow-up at the department of internal medicine of Messina for recurrence of ascitic effusion and with feedback to the hypereosinophilia blood count (550 cells) that came to my attention in February 2021 for poorly productive cough and dyspnea from minimal efforts. I was doing spirometry with bronchoversibility tests that show slight restrictive deficit and not significant broncoreversibility test, with the following values:
- FEV1 pre e post: 74% theoretical
- PEF pre e post: 70% theoretical
- FVC: 75% theoretical
- FEV1/FVC: 102% theoretical
- FEF25-75: pre: 53% theoretical post: 62% theoretical (+16%)
- Bronchial reversibility not strong after 400mcg of salbutamol (little variation, of FEF25-75 no variation of FEV1 and PEF).
In addition to the spirometry, I carried out ultrasound control with the presence of pleural effusion prevalent on the right (2 intercostal spaces) and ascitic effusion that explain the spirometry findings. After the first visit, the patient carried out inhalation therapy with Aclidinium bromide BID and mepolizumab for hypereosinophilia found at the blood count (550 eosinophils) with subsequent clinical and spirometry control in June 2021which showed:
- FEV1 87: % theoretical (+13%)
- PEF: 70% theoretical
- FVC: 87% theoretical (+12%)
- FEV1/FVC: 104% theoretical (+2%)
- FEF25-75: 69% theoretical (+16%)
- Monitoring of eosinophil ematic counts and FENO with VIATOM portable device, showed respectively: 480 eosinophils (-70 cell.) and 25 ppb, which still indicated hypereosinophilic asthma that was not well controlled.
- At ecographic examination no pleural and ascitic effusion.
At the subsequent control carried out 7 months after therapy with biologic and aclidinium bromide, the spirometry showed a clear improvement in absolute values and the patient reported net well-being with disappearance of the previously reported symptomatology and reduction of FENO values (14ppb vs. 25 ppb) and eosinophilic count (300 vs. 480 cell.).
DISCUSSION AND CONCLUSIONS
In light of the above clinical history, the diagnosis of overlap syndrome (ACO) associated with autoimmune disease of the biliary tract was formulated. It represents the only clinical case in the literature that associates the two diseases and that allows to see the effectiveness of the biological in both pathologies. The mechanism of action of Anti-IL-5 would play a role in blocking the production of eosinophils and which will have to be demonstrated in other clinical studies.
1.Role of Biologics in Asthma Mary Clare McGregor1, James G. Krings1, Parameswaran Nair2, and Mario Castro1 1 Division of Pulmonary and Critical Care, Department of Medicine, Washington University School of Medicine, St. Louis, Missouri; and 2 Division of Respirology, Department of Medicine, St. Joseph’s Healthcare Hamilton, McMaster University, Hamilton, Ontario, Canada.
2. Mepolizumab in eosinophilic disorders J Pablo Abonia1 and Philip E Putnam2,† 1Division of Allergy and Immunology, Cincinnati Children’s Hospital Medical Center, University of Cincinnati College of Medicine, 3333 Burnet Avenue, ML2010, Cincinnati, OH 45229-3039, USA 2Division of Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Cincinnati Children’s Hospital Medical Center, University of Cincinnati College of Medicine, 3333 Burnet Avenue, ML2010, Cincinnati, OH 45229-3039, USA
3. Mepolizumab in the treatment of eosinophilic chronic obstructive pulmonary disease Takudzwa Mkorombindo Mark T Dransfield Lung Health Center, Division of Pulmonary, Allergy and Critical Care Medicine, Department of Medicine, University of Alabama at Birmingham, Birmingham, AL, USA
4. Mepolizumab for Eosinophil-Associated COPD: Analysis of METREX and METREO