CASE REPORT |
https://doi.org/10.5005/jp-journals-10057-0211 |
Primary Intestinal Lymphangiectasia leading to Protein losing Enteropathy
1–12Department of Internal Medicine, Mahatma Gandhi Hospital (MGH), Mahatma Gandhi University of Medical Sciences & Technology (MGUMST), Jaipur, Rajasthan, India
Corresponding Author: Deepak Gupta, Department of Internal Medicine, Mahatma Gandhi Hospital (MGH), Mahatma Gandhi University of Medical Sciences & Technology (MGUMST), Jaipur, Rajasthan, India, Phone: +91 07597965979, e-mail: deepakguptamd@gmail.com
Received on: 12 May 2022; Accepted on: 11 August 2022; Published on: 22 February 2023
ABSTRACT
Protein losing enteropathy (PLE) describes a diverse group of disorders associated with excessive loss of serum proteins into the gastrointestinal (GI) tract. This excess protein loss leads to hypoproteinemia and may be manifested by edema, ascites, and malnutrition. This case of a rare condition, primary intestinal lymphangiectasia leading to protein loss, is being reported to highlight the clinical features and presentation of these disorders as they are easily detectable and treatable, and many can be cured.
How to cite this article: Sharai V, Gupta D, Kalia A, et al. Primary Intestinal Lymphangiectasia leading to Protein losing Enteropathy. J Mahatma Gandhi Univ Med Sci Tech 2022;7(2):52-54.
Source of support: Nil
Conflict of interest: None
Patient consent statement: The author(s) have obtained written informed consent from the patient for publication of the case report details and related images.
Keywords: Ascites, Edema, Hypoproteinemia, Intestinal lymphangiectasia, Malnutrition, Medium chain triglycerides.
INTRODUCTION
Protein losing enteropathy (PLE), sometimes referred to as protein-losing gastroenteropathy, is an unusual cause of hypoproteinemia and is characterized by the shedding of large quantities of protein from the GI mucosa. PLE may result from a wide variety of etiologies and can be both a diagnostic and therapeutic challenge to the practicing physician/gastroenterologist. The clinical presentation of PLE may also be complicated by micronutrient deficiencies related to the underlying etiology of the PLE. PLE is generally considered to be a rare condition; however, given a lack of systematic screening and a wide variety of causes of hypoalbuminemia, its prevalence is poorly understood.
Although some patients with PLE present with severe GI symptoms, such as diarrhea, which can take on a secretary character, it is important to recognize that not all patients suffering from PLE will exhibit overt GI symptoms. In fact, the key clinical characteristic of PLE is symptomatic hypoalbuminemia which manifests most commonly as edema. Other clinical manifestations generally reflect the underlying disease responsible for PLE.1,2
CASE DESCRIPTION
A 60-year-old male patient presented to the hospital with complaints of loose stools (on-off) for 4 years and swelling in bilateral (B/L) lower and upper limbs for the last 2 years (Fig. 1).
Fig. 1: Showing pitting edema in B/L lower limbs
The swelling was also seen in his genital area so much that it was extremely painful to the patient for the last 3 months. Then the swelling kept on progressing further leading to difficulty or restriction in carrying out his daily activities.3
Examination revealed a conscious, oriented patient with all vitals within normal limits. On examination, the patient had significant severe edema on all four limbs and on genitalia with no further abnormality in any other system.4
A step-by-step approach via investigations to rule out the causes of edema was followed (Table 1).
| Albumin | 3.8 gm/dL |
| Globulin | 1.2 gm/dL |
| Endoscopy, colonoscopy, immunoglobulin G, tissue transglutaminase | Normal |
| Liver and renal function test | Normal |
| Ultrasound whole abdomen, urine routine | Normal |
| Antinuclear antibodies, viral markers, rheumatoid arthritis factor | Negative |
| Tc colloid lymphoscintigraphy | Intestinal lymphangiectasia |
| Jejunal biopsy | Dilated lymphatics |
Since all other causes of edema were ruled out, protein-losing gastroenteropathy, a rare cause and diagnosis of exclusion needed to be considered. Therefore, technetium (Tc) colloid lymphoscintigraphy was done which confirmed the presence of intestinal lymphangiectasia which was of primary type as no obstruction to lymphatics was involved. Further to confirm the diagnosis, a jejunal biopsy was done which showed dilated lymphatics and no mucosal ulceration (Fig. 2).
Fig. 2: Jejunal biopsy showing dilated lymphatics
A low-fat diet enriched with medium chain triglycerides, which do not require lymphatic transport and therefore do not stimulate lymph flow, decreased the enteric protein loss leading to significant improvement in the patient’s symptoms within 4 weeks.5,6
Diuretics and supportive stockings along with scrotal support were also given.
Exercise and adequate ambulation were encouraged to reduce the risk of venous thrombosis. Skincare was also taken care of meticulously to prevent skin breakdown and cellulitis.
DISCUSSION
A three-diagrammatic representation of “PLE syndrome” diseases [lymphangiectasia, lymphangitis, crypt lesions, inflammatory bowel disease (IBD), and mucosal ulceration] and the effect of Starling forces on intestinal osmolality and the interstitial-lumenal oncotic gradient is shown in Figure 3. Protein leaks into the small intestine passively, via an oncotic gradient from the interstitium to the lumen. If osmotic pressures equilibrate, a reduction in the passive leak is expected. When the intestinal lumen has a higher oncotic pressure than the interstitium, such as postprandially, the gradient may be reversed, depending on the underlying pathology. Top—magnification of lymphatic capillary structure, showing overlapping endothelial cells that act as flap-like doors allowing interstitial fluid to enter, while preventing lymph exit. IBD, PLE.
Fig. 3: Diagrammatic representation of “PLE syndrome”
Because PLE is a rare disease with a variety of seemingly disparate causes, there are limited data on its optimal treatment. As such, no single treatment reliably improves PLE in all patients. A core principle is to treat the underlying disease which, if successful, should generally result in improvement in the PLE. Fortunately, most causes of PLE can be readily diagnosed and treated.
A number of PLE-specific strategies have been described and include dietary, pharmacological, or surgical interventions.
Direct replacement of serum albumin by infusion is not a useful long-term strategy as it provides only short-term benefits, is expensive, and does not reverse the underlying pathophysiology. In the acute setting, albumin infusion may help patients suffering from the severe third spacing of fluid due to marked hypoalbuminemia as a bridge to more durable therapies. Supportive measures to avoid complications resulting from fluid retention contribute meaningfully to PLE patients’ quality of life.
To conclude, the patient described in this case represents a typical case of PLE with a GI etiology. It highlights the need to recognize PLE as a cause of unexplained hypoalbuminemia. PLE can occur in the context of a myriad of diseases ranging from primary GI mucosal disorders to malignancies, lymphatic disorders, to congenital heart disease. While the evidence regarding the management of PLE is limited, treatment primarily focuses on the underlying disease with the addition of supportive measures to manage complications such as edema.
ORCID
Anchin Kalia https://orcid.org/0000-0001-8869-9351
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