Severe Alveolar Hemorrhage – What’s in it for the Gastroenterologist?

Background: Alveolar hemorrhage is a potentially life-threatening condition which is usually managed by the pulmonologist. When considering its etiology, there is a rare association that sets the disease into the hands of the gastroenterologist. Case presentation: We report the case of a 48 year-old female who was admitted to the intensive care unit for severe anemia and hemoptysis. On imaging, diffuse pulmonary infiltrates suggestive of alveolar hemorrhage were detected and a diagnosis of pulmonary hemosiderosis was made. She received cortisone therapy and hematologic correction of anemia, with slow recovery. In search of an etiology for the pulmonary hemosiderosis, an extensive workup was done, and celiac disease specific serology was found positive. After confirmation of celiac disease by biopsy, a diagnosis of Lane-Hamilton syndrome was established. The patient was recommended a gluten-free diet and at 6 months follow-up, resolution of anemia and pulmonary infiltrates were observed. Conclusion: Although the association is rare, celiac disease should be considered in a patient with idiopathic pulmonary hemosiderosis. In our case, severe anemia and alveolar infiltrates markedly improved with glucocorticoids and gluten-free diet. Abbreviations: APTT: activated partial thromboplastin time; BAL: bronchoalveolar lavage; CD: celiac disease; Cd: crypt depth; GFD: gluten-free diet; GI: gastrointestinal; IEL: intraepithelial lymphocyte; INR: international normalized ratio; IPH: idiopathic pu

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Serodiagnosis of Celiac Disease in Children Referred for Evaluation of Anemia: A Pediatric Hematology Unit’s Experience.

Blood ◽

10.1182/blood.v106.11.1665.1665 ◽

2005 ◽

Vol 106 (11) ◽

pp. 1665-1665

Author(s):

R.K. Marwaha ◽

Deepak Bansal ◽

Amita Trehan ◽

Akash Patel

Keyword(s):

Celiac Disease ◽

Small Bowel ◽

Tissue Transglutaminase ◽

Gluten Free Diet ◽

Gluten Free ◽

Indian States ◽

Duration Of Symptoms ◽

Pulmonary Hemosiderosis ◽

Proximal Small Bowel ◽

The Mean

Abstract Celiac disease (CD) is a malabsorptive disorder wherein the proximal small bowel mucosa is damaged as a result of dietary exposure to gluten. Children with intractable diarrhea and failure to thrive are diagnosed with relative ease. Diagnosis can however be challenging and is often delayed when children present with ‘difficult to treat anemia’, without overt gastrointestinal manifestations. The case records of 77 patients with CD were scrutinized retrospectively. Diagnosis was established with serology (tissue transglutaminase-IgA assay) in 46 (59.7%), serology along with small bowel mucosal biopsy in 23 (29.9%) and with biopsy alone in the remaining 8 (10.4%). All children belonged to the predominantly wheat consuming northern Indian states. The mean age at presentation was 99.1±34.8 months (median: 102, range: 22–168). Males outnumbered females in a ratio of 1.96:1. The mean duration of symptoms was 41±31.2 months (median: 36, range: 1–132). The overwhelming majority, i.e., 75 (97.4%) children had anemia (Hemoglobin <11 g/dL). Mean hemoglobin (Hb) was 7.0±2.2 g/dL (median: 7.2, range: 2.3–12.5). 52 (67.5%) had received iron supplements for sufficient lengths, without benefit. The red cell morphology was microcytic hypochromic in 37 (48%) and dimorphic in 33 (42.9%). A history of diarrhea was not forthcoming in 32 (41.6%) cases. 59 (76.6%) were malnourished, with a weight less than 80 % of expected for the age and 30 (39 %) were stunted, with a height falling below the 90% of expected. Two children had skin bleeds secondary to coagulopathy, due to Vitamin K malabsorption. In another 2, recurrent anemia was attributed to pulmonary hemosiderosis; further investigations for secondary causes unearthed CD. All children were initiated on an austere gluten free diet, along with iron and folic acid supplements for the initial 6–9 months. Mean duration of follow was 17.7±20.9 months. Improvement was perceptible within days of initiating gluten free diet. Of the 38 (49.4%) children who had a follow up of a year or longer, the mean Hb at the last visit had risen to 12.9±1.2 g/dL. Conclusions: Hematologists need to be aware of the mono-symptomatic presentation of CD with anemia. The typical period of presentation of CD is described to be between 6 mo and 2 yr of age. Prolonged duration of symptoms and a diagnosis at a relatively older age is striking in the index study. In a suggestive clinical background, identification of CD with serodiagnosis alone, without resorting to small bowel biopsy is increasingly gaining acceptance, as the specificity of newer serological assays is 95–98%. This is particularly true in tropical countries, where some degree of flattening of villi may be attributed to malnutrition and or infections, such as rotavirus enteritis, Giardia lamblia, or tropical sprue. A biopsy may be misleading in such cases. Heightened awareness is essential to identify CD at an early age, especially, in children in whom anemia is the dominant manifestation. The benefits of gluten free diet are apparent with the rise in hemoglobin and the improvement in growth parameters are gratifying both for physicians and the caretakers.

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Hemoptysis in patients of celiac disease with disproportionately severe anemia: tip of the iceberg?

Multidisciplinary Respiratory Medicine ◽

10.4081/mrm.2013.501 ◽

2013 ◽

Vol 8 ◽

Author(s):

Kamal Kumar Singhal ◽

Ashok K. Janmeja ◽

Rakhee Sodhi ◽

Rajpal S. Punia

Keyword(s):

Celiac Disease ◽

Refractory Anemia ◽

Deficiency Anemia ◽

Severe Anemia ◽

Gluten Free ◽

Therapeutic Implications ◽

Pulmonary Hemosiderosis ◽

Disease Entities ◽

Gastrointestinal Complaints ◽

Idiopathic Pulmonary Hemosiderosis

Idiopathic Pulmonary Hemosiderosis (IPH) is characterized by the triad of iron deficiency anemia, pulmonary infiltrates and haemoptysis with no recognizable cause. Since the first description of its association with Celiac Disease (CD) by Lane and Hamilton in 1971, only a few isolated cases have been reported in literature. Although it has been considered an uncommon association of two disease entities, recent reports indicate that prevalence of celiac disease is as high as one percent. Further, individually both celiac disease and IPH are known to present as refractory anemia only. We are reporting a young adult with Lane Hamilton Syndrome, who realized that he was having significant gastrointestinal complaints only when they disappeared on gluten free diet (GFD). This case report reiterates the fact that celiac disease should be considered in all patients of IPH because of the therapeutic implications. Further on review of literature, we believe that covert hemoptysis may be responsible for disproportionately severe anemia in patients of celiac disease. Thus, prevalence of this association may be more than currently believed. Further research in this regard may improve our understanding of pathogenesis of celiac disease.

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Benefit of gluten-free diet in idiopathic pulmonary hemosiderosis in association with celiac disease

Pediatric Pulmonology ◽

10.1002/ppul.21357 ◽

2010 ◽

Vol 46 (3) ◽

pp. 302-305 ◽

Cited By ~ 21

Author(s):

Gulshan R. Sethi ◽

Kamal K. Singhal ◽

Amarender S. Puri ◽

Mukta Mantan

Keyword(s):

Celiac Disease ◽

Gluten Free Diet ◽

Gluten Free ◽

Pulmonary Hemosiderosis ◽

Idiopathic Pulmonary Hemosiderosis

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Extracorporeal Membrane Oxygenation for Diffuse Alveolar Hemorrhage Caused by Idiopathic Pulmonary Hemosiderosis: A Case Report and a Review of the Literature

Journal of Pediatric Intensive Care ◽

10.1055/s-0039-1679904 ◽

2019 ◽

Vol 08 (03) ◽

pp. 181-186 ◽

Cited By ~ 2

Author(s):

Shotaro Matsumoto ◽

Satoshi Nakagawa

Keyword(s):

Extracorporeal Membrane Oxygenation ◽

Rescue Therapy ◽

Diffuse Alveolar Hemorrhage ◽

Alveolar Hemorrhage ◽

Membrane Oxygenation ◽

Pulmonary Hemosiderosis ◽

Threatening Condition ◽

Life Threatening ◽

Systemic Anticoagulation ◽

Idiopathic Pulmonary Hemosiderosis

AbstractDiffuse alveolar hemorrhage (DAH) is a life-threatening condition presenting with hemoptysis, anemia, and diffuse radiographic pulmonary infiltrates; it causes acute respiratory failure. Idiopathic pulmonary hemosiderosis (IPH) is a rare cause of DAH occurring predominantly in children. Bleeding is often considered to be a contraindication for extracorporeal membrane oxygenation (ECMO) due to systemic anticoagulation. We present an 8-year-old girl with DAH caused by IPH. Unfractionated heparin was administered to maintain an activated clotting time of 150 to 180 seconds. The DAH resolved with immunosuppressive therapy, and the patient survived to decannulation. ECMO may be applied as a rescue therapy for DAH even with systemic anticoagulation.

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