Intestinal Amyloidosis in Common Variable Immunodeficiency and Rheumatoid Arthritis

Meira, Tânia; Sousa, Rita; Cordeiro, Ana; Ilgenfritz, Raquel; Borralho, Paula

doi:10.1155/2015/405695

Cited by 5 publications

(6 citation statements)

References 15 publications

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“…Secondary amyloidosis can occur in response to any chronic inflammatory state, with 40% of cases related to rheumatoid arthritis [4]. Other known causes include periodic fever syndromes, such as familial Mediterranean fever, inflammatory bowel disease, lymphoproliferative disorders, and tuberculosis, but very few cases have been described in association with CVID or other hypogammaglobulinemia in the last 25 years [1][2][3][4][8][9][10][11][12][13][14][15][16].…”

Section: Discussionmentioning

confidence: 99%

Secondary Amyloidosis and Common Variable Immunodeficiency: A Rare Association

Lopes¹,

Peixoto²,

Antunes³

et al. 2022

Cureus

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Common variable immunodeficiency (CVID) is a disease characterized by severe antibody deficiency due to impaired B cell differentiation. It represents the most common form of primary immunodeficiency in children and adults, and its clinical manifestations include recurrent infections and chronic lung disease, gastrointestinal infections, and autoimmunity. Here, we present the case of a 47-year-old female patient with a history of CVID and recurrent Campylobacter jejuni bacteremia. She was undergoing biweekly administration of intravenous immunoglobulin for over 15 years. During hospitalization rapidly progressive oliguric renal failure was observed in association with anasarca and nephrotic syndrome. Bilateral nephromegaly was noted on an abdominal pelvic computed tomography scan. Renal biopsy was consistent with amyloidosis, and serum amyloid A protein was elevated. The diagnosis of AA amyloidosis secondary to CVID was made. The patient was started on hemodialysis and weekly intravenous immunoglobulin administration with favorable clinical outcomes.

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Section: Discussionmentioning

confidence: 99%

Secondary Amyloidosis and Common Variable Immunodeficiency: A Rare Association

Lopes¹,

Peixoto²,

Antunes³

et al. 2022

Cureus

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“…Less often, patients present with bloody diarrhea associated with fever and abdominal pain or cramping. [17][18][19] Recurrent diarrhea may be associated with muscle weakness, electrolyte disturbances, or peripheral paresthesia in the lower extremities. 16 Differential diagnosis requires the exclusion of infectious and parasitic causes of diarrhea, including Salmonella, Shigella, Campylobacter, and toxin A for Clostridium difficile analysis.…”

Section: Diarrheamentioning

confidence: 99%

“…Additionally, Congo red staining allowed for the confirmation of amyloidosis by the detection of green birefringence on amyloid depositions in the lamina propria of the colonic mucosa. 16,18,19 The mechanism behind the presentation of diarrhea in patients with amyloidosis is not fully understood. It is generally suggested that diarrhea arises due to motility disturbances of the gastrointestinal tract which are caused by autonomic neuropathy and intestinal inflammation.…”

Section: Diarrheamentioning

confidence: 99%

Intestinal amyloidosis: Clinical manifestations and diagnostic challenge

Talar-Wojnarowska¹,

Jamroziak²

2021

Adv Clin Exp Med

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Amyloidosis is a heterogeneous group of diseases in which the extracellular deposition of abnormal fibrillar proteins disrupts tissue structure and function. Intestinal involvement is a very rare manifestation of amyloidosis compared to the most affected organs, the heart and kidneys. Damage of the gastrointestinal tract may be the only manifestation of amyloidosis, or -more often -is a component of the involvement of several organs in systemic amyloidosis. Any part of the digestive tract can be involved; however, the small bowel is the most affected part, followed by the colon. Intestinal amyloidosis is characterized by a heterogeneous clinical picture, with weight loss, chronic diarrhea, abdominal pain, intestinal bleeding, or pseudo-obstruction. Endoscopic findings are characterized by a fine granular appearance, erosions, ulcerations, mucosal friability, multiple protrusions, or tumor-like lesions. Pathologic examination allows for a definitive diagnosis using Congo red staining and a positive sample with apple-green birefringence. The disease can easily be misdiagnosed with several other diseases of the digestive tract and lead to diagnostic challenges in clinical practice. Further, the amyloid colonic deposition may mimic inflammatory bowel disease, malignancy, ischemic colitis, and collagenous colitis. Therefore, gastroenterologists need to include amyloidosis in their diagnostic work-up.

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“…Similarly, several primary immunodeficiencies have been associated with AA amyloidosis, including common variable immunodeficiency, 121‐130 X‐linked agammaglobulinemia, 131‐134 cyclic neutropenia, 135 chronic granulomatous disease, 84,136,137 hyperimmunoglobulin E syndrome, 138 and leucocyte adhesion deficiency 139 …”

Section: Infections Associated With Aa Amyloidosismentioning

confidence: 99%

Infections and AA amyloidosis: An overview

et al. 2021

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Background Amyloidoses are a heterogeneous group of systemic diseases characterised by extracellular accumulation of insoluble amyloid fibrils derived from unfolded proteins. Inflammatory (AA) amyloidosis can complicate various inflammatory disorders that are associated with a sustained acute phase response and serum amyloid A (SAA) protein overproduction. Chronic infections were the first recognised cause of amyloidoses. However, with the better management of underlying diseases, the frequency of AA amyloidosis is decreasing. Purpose The aim of this overview was to discuss the several infections associated with AA amyloidosis and the relative frequency of infections as aetiological factors. Methods A search of the literature was performed using the PubMed database using the MeSH terms “Amyloidosis” and “Infections,” from inception to December 31st, 2019. Articles written in other languages than English or French were excluded. Results The frequency of AA amyloidosis secondary to infections decreased from more than 50% to less than 20% after the 2000s, with a parallel increase in the frequency of AA amyloidosis secondary to inflammatory diseases and to an unknown cause. Conclusion Whereas new antibiotics have been developed and sanitary conditions are better, infections still represent 5%‐30% of the causes of AA amyloidosis, including in developed countries. These data argue for better screening of chronic infections to prevent AA amyloidosis and the development of new strategies to manage recurrent infections.

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Intestinal Amyloidosis in Common Variable Immunodeficiency and Rheumatoid Arthritis

Cited by 5 publications

References 15 publications

Secondary Amyloidosis and Common Variable Immunodeficiency: A Rare Association

Secondary Amyloidosis and Common Variable Immunodeficiency: A Rare Association

Intestinal amyloidosis: Clinical manifestations and diagnostic challenge

Infections and AA amyloidosis: An overview

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