Is it worth investigating splenic function in patients with celiac disease?

Table 1 Case reports of hyposplenism-related infections in patients with celiac disease

Ref.	No. of cases	Type of infection	Supplementary findings
Corazza et al[23]	1	Pneumoccoccal pneumonia	Splenic atrophy
Matuchansky et al[24]	2	Pneumococcal pneumonia, infectious pericarditis	Splenic atrophy, MLNC
O’Donoghue[25]	1	Pneumococcal septicemia	Splenic atrophy
Logan et al[26]	2	Pneumococcal meningitis, septicemia by Salmonella	Splenic atrophy
Stevens et al[27]	3	Lung abscesses by Staphyloccus and Klebsiella	Splenic atrophy
Howat et al[28]	2	Fatal chest infection, septicemia	Splenic atrophy, MLNC
Harmon et al[29]	1	Septicemia by Klebsiella	Splenic atrophy

MLNC: Mesenteric lymph node cavitation.

Table 2 Traditional polysaccharide and new conjugate anti-pneumococcal vaccines used in the prophylactic management of asplenic/hyposplenic patients

Vaccine	Brand name	Structure	Mechanism	Serotype	Indication
PPV23	Pneumovax®	Polysaccharide	T-cell independent	1, 2, 3, 4, 5, 6B, 7F, 8, 9N, 9V, 10A, 11A, 12F, 14, 15B, 17F, 18C, 19A, 19F, 20, 22F, 23F, 33F	Asplenic or hyposplenic adults Asplenic or hyposplenic children > 5 yr
PCV13	Prevnar®	Protein-conjugate (CRM197 protein)	T-cell dependent	1, 3, 4, 5, 6A, 6B, 7F, 9V, 14, 18C, 19A, 19F, 23F	Asplenic or hyposplenic children < 5 yr

PPV: Pneumococcal polysaccharide vaccine; PCV: Protein-conjugate pneumococcal vaccine.

Table 3 Celiac patients in whom splenic function should be assessed

Patients with complications (RCD, UJI, EATL, collagenous sprue)

Patients with concomitant autoimmune disorders

Patients with old age at diagnosis

Patients with previous history of major infections/sepsis and/or thromboembolism

Patients with mesenteric lymph node cavitation and/or splenic atroph

EATL: Enteropathy-associated T-cell lymphoma; RCD: Refractory celiac disease; UJI: Ulcerative jejuno-ileitis.