Celiac Disease Testing Recommended for Patients with Idiopathic Pulmonary Hemosiderosis

Celiac.com 05/18/2022 - Idiopathic pulmonary hemosiderosis causes diffuse alveolar hemorrhage, but the mechanics of the cause remains unknown. 

Alveolar hemorrhage is a "life-threatening disorder characterized clinically by the presence of hemoptysis, falling hematocrit, diffuse pulmonary infiltrates and hypoxemic respiratory failure. Diffuse alveolar hemorrhage should be considered a medical emergency due to the morbidity and mortality associated with failure to treat the disorder promptly."

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The occurrence of idiopathic pulmonary hemosiderosis and celiac disease together has been noted in both children and adults, and is also known as Lane-Hamilton syndrome. 

A team of researchers recently set out to compare demographics, clinical and radiologic findings, treatment, and outcomes between adult patients with idiopathic pulmonary hemosiderosis and Lane-Hamilton syndrome.

The research team included Biplab K. Saha, Praveen Datar, Alexis Aiman, Alyssa Bonnier, Santu Saha, and Nils T. Milman.

They are variously affiliated with the Department of Clinical Biochemistry, University College Zealand in Næstved, Denmark; the department of Internal Medicine, New York Institute of Technology College of Osteopathic Medicine at Arkansas State University in Jonesboro, USA; the Pulmonary and Critical Care Medicine, Ozarks Medical Center · West Plains, USA; the center for Critical Care, Goldfarb School of Nursing at Barnes Jewish College · Saint Louis, USA; and the Internal Medicine, Saha Clinic in Narail, Bangladesh.

For their systematic review of the literature, the team used proper search parameters to identify relevant articles in multiple databases. Their final review included a total of 60 studies reporting 65 patients. Forty-nine of these patients had idiopathic pulmonary hemosiderosis, while 16 had Lane-Hamilton syndrome. 

Thirteen of twenty-two patients screened, nearly sixty percent, were positive for anti-celiac antibodies. Patients with Lane-Hamilton syndrome showed earlier symptom onset and diagnosis of idiopathic pulmonary hemosiderosis, though both groups showed an average delay in diagnosis of about one year.

Lane-Hamilton syndrome patients were most likely to show the classic symptom triad, although only one in five patients in the Lane-Hamilton syndrome group showed any significant gastrointestinal symptoms at the time of idiopathic pulmonary hemosiderosis diagnosis. 

A gluten-free diet alone was effective in the majority of patients. Fewer patients in the Lane-Hamilton syndrome cohort received systemic corticosteroid than the idiopathic pulmonary hemosiderosis cohort. 

The recurrence and mortality in patients with Lane-Hamilton syndrome appear to be less than in the idiopathic pulmonary hemosiderosis cohort. 

The results show that one in four adult patients with idiopathic pulmonary hemosiderosis has celiac disease. Patients with Lane-Hamilton syndrome may show milder effects than patients without celiac disease. 

Based on these results, the researchers are recommending that all idiopathic pulmonary hemosiderosis patients receive serologic screening for celiac disease.

Read more at Cureus.com 14(3): e23482