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

Abstract

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.

Lane Hamilton Syndrome

“…Among few published cases of Lane Hamilton syndrome, six cases are Indian with male predominance and being diagnosed very late similar to our case [3,4]. Furthermore, suspicion for both components of the syndrome is very low despite higher disease prevalence (1%) of CD in North India [2].…”

Hemorrhagic Events in Adult Celiac Disease Patients. Case Report and Review of the Literature

“…In the cases reviewed, different clinical situations explained the development of hemorrhagic events, such as coagulopathy due to vitamin K malabsorption [4,15,20,23,32,44,45,47], celiac crisis [8,13], LHS [14,15,17,18,[33][34][35][36][37][38][39][40], portal hypertension [23], or digestive tumors [19,21,25,41].…”

“…In 1971, the IPH presence was described in a 23-year-old man with idiopathic steatorrhea (LHS) [63]. The pulmonary symptomatology in LHS starts usually in childhood, and only 25% of all patients have an adult onset [14,35]. Xi-Yuan et al reviewed the literature for IPH and identified 37 patients (12 women, 24 men), out of whom 5 had CD [64].…”

“…Also, in eight cases the improvement was established after biopsy, when histological amelioration/ restoration under GFD was noted [9, 18, 26-28, 32, 49, 51]. In other cases, different parameters were described to support the favorable response under GFD, such as: clinical improvement [8, 10, 12-14, 17, 24, 35, 36, 47], increase in hemoglobin levels [12-14, 35, 36, 37, 52, 53], pulmonary recovery [12,14,36] and weight gain [13,14,46,[52][53][54].…”

“…Besides coagulopathy, diffuse alveolar hemorrhage with idiopathic pulmonary hemosiderosis (IPH) has been described in CD, an association also known as the Lane Hamilton syndrome (LHS) [14,15,17,18,[33][34][35][36][37][38][39][40] and presenting with hemoptysis. Moreover, intestinal tumors [19,21,25,41] or variceal bleeding [23] can determine digestive hemorrhage.…”