Classification of Intestinal Lymphangiectasia with Protein-Losing Enteropathy: White Villi Type and Non-White Villi Type

Abstract

Background/Aims: We classified intestinal lymphangiectasia (IL) into two categories, the white and non-white villi types, and evaluated their clinical characteristics and therapeutic responses. Methods: Of the 988 patients who underwent double-balloon enteroscopy, 14 consecutive patients (7 men and 7 women, median age at onset 34 years) were enrolled with immunohistochemically confirmed IL with protein-losing enteropathy. Results: Enteroscopically the white villi type (n = 8) showed white plaques and white-tipped villi were scattered in the small bowel, while non-white villi type (n = 6) showed that apparently normal but under more detailed observation, low and round villi with a normal color were diffused. The serum albumin levels and fecal E₁-antitrypsin clearance before treatment were significantly worse in the non-white villi type (p = 0.017 and 0.039, respectively), whereas the serum immunoglobulin A and M levels were significantly lower in the white villi type (p = 0.010 and 0.046, respectively). At gastroscopy, a non-cirrhotic snakeskin appearance was significantly observed in the non-white villi type (p = 0.015). The corticosteroid response was better in the non-white villi type (p = 0.015). Conclusion: Two distinct subgroups were found in IL. This classification was useful in pathophysiological clustering and in predicting the therapeutic response. i 2014 S. Karger AG, Basel

In vivo characterization of abnormalities in small-bowel diseases using probe-based confocal laser endomicroscopy

“…We proposed a classification of intestinal lymphangiectasia into white villi type (the former lamina propria dominant type) and non-white villi type (the latter deeper layer dominant type) according to the enteroscopic findings, which clustered its manifestations and severity. Enteroscopically, the non-white villi type showed that apparently normal, but under more detailed observation, low and round villi with a normal color, were diffused 17 . In two of three patients with non-white villi type intestinal lymphangiectasia, pCLE was able to detect lymphangiectasia, while detection did not occur in the remaining patient who was diagnosed only by autopsy, showing predominantly subserosal lymphangiectasia accompanied by marked fibrosis in the submucosal layer (data not shown).…”

Protein-losing Enteropathy Caused by Intestinal or Colonic Lymphangiectasia Complicated by Sporadic Lymphangioleiomyomatosis: A Report of Two Cases

“…Ohmiya et al noted that the white villi type (white plaques and white-tipped villi) as reflecting the pathological feature of dilated lymphatic vessels in the lamina propria, while the non-white villi type represented the dilation of the lymphatic vessels in the submucosal layer or deeper (5). In our study, Patient 2 had similar white villi, confirming the dilation of the lymphatic vessels in the lamina propria.…”

CHAPLE syndrome uncovers the primary role of complement in a familial form of Waldmann's disease

“…Perhaps, the variable course of PLE in the natural history of PIL is not unique for CHAPLE patients. Some of the reported PIL cases had spontaneous recovery of their symptoms, remaining well for prolonged period, with relapses over time …”

“…Individual reports have described thrombotic events among patients with PLE caused by various etiologies, including PIL, PLVAP deficiency, and lupus‐related enteropathy . Among several predisposing factors, the theory that loss of anti‐coagulatory molecules such as anti‐thrombin III, protein C, protein S, and plasminogen in the gut produces a thrombophilic state in PLE has gained specific interest .…”

“…However, the long-term prognosis is unpredictable in a given patient because abrupt relapses are reported even following periods of prolonged remission. 88,89 There appears to be no genotype-phenotype correlation in CHAPLE, as suggested by varying levels of disease severity among siblings who have the same particular mutation. 85 It is also not uncommon that a family screen may identify individuals with homozygous mutations who are asymptomatic per self-or parental-reports.…”