Lymphogranuloma venereum: a rare and forgotten cause of rectal stricture formation

Case report

This 56-year-old White British, man who has sex with men presented in 2010 with altered bowel habit, rectal bleeding and tenesmus. Sigmoidoscopy revealed a 3-mm ulcerative lesion with histology consistent with ulcerative colitis (Figure 1(a)).

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Figure 1.

(a) Endoscopy, May 2013, suggestive of ongoing proctitis up to 12 cm from the anal orifice. (b, c) Endoscopy, March 2014, showing confluent ulceration from anus to 10 cm. (d–g) Endoscopy, October 2015 and November 2015, showing a benign-appearing stenosis in the rectum at about 3–4 cm. (h, i) Endoscopy, February 2016, showing stricture at 4 cm from the anal verge.

After a two-year symptom-free interval, he was readmitted with worsening symptoms despite oral and rectal mesalazine and 40 mg prednisolone. His symptoms progressed on budesonide, mesalazine orally and mesalazine enemas. Serial sigmoidoscopies reflected progressive proctitis. Histology showed severe distortion of gland crypt architecture with moderate goblet cell depletion and an active chronic proctitis. He was started on acetarsol suppositories and azathioprine 150 mg od. His symptoms never fully settled.

In March 2014, he was referred to haematology when sigmoidoscopy revealed confluent ulceration from anus to 10 cm (Figure 1(b) and (c)) and histology showed a dense infiltrate of polymorphous plasma cells and large transformed lymphoid cells in the lamina propria. These findings were eventually ascribed to Epstein Barr Virus (EBV) driven B-cell lymphoid proliferation, secondary to azathioprine rather than lymphoma. His serum EBV level was 970 copies/ml. Azathioprine was stopped.

In September 2014, he was readmitted with rectal bleeding, pyrexia and night sweats. Abdominal radiography was normal and repeat stool culture was negative. Ulceration seen on sigmoidoscopy was now more in keeping with ‘Crohn’s’ colitis. At this stage a rectal dual nucleic acid amplification test (NAAT) was performed and found to be positive for Chlamydia. This was subsequently confirmed as lymphogranuloma venereum (LGV) (TaqMan^™ in-house quantitative polymerase chain reaction [qPCR], Applied Biosystems, Colindale, UK). The test performed detected the pmpH gene found in all LGV serovars. Specific typing was not carried out. Histology did not show any specific features of LGV and reflected nonspecific inflammation only.

The last sexual intercourse reported was in San Francisco in 2010, shortly before his symptoms began. Due to the proctitis, he had been unable to receive anal intercourse since that time. He tested human immunodeficiency virus-1 (HIV-1) antibody positive. His CD4 cell count was 635 cells/μl with a CD4:CD8 ratio of 0.28 due to marked serum lymphocytosis. He had a fully susceptible virus and a HIV viral load of 4076 copies/ml. Serum cytomegalovirus (CMV) PCR was undetectable and EBV PCR was now <500 copies/ml.

Despite LGV treatment with three weeks of doxycycline 100 mg bd, excellent HIV suppression and steady immune reconstitution with correcting of his CD4:CD8 ratio, he experienced ongoing diarrhoea and rectal pain. Repeated rectal NAATs, syphilis testing and PCR for a variety of other microorganisms including microspora PCR was negative. Consultation with national experts in LGV resulted in retreatment with doxycycline followed by ofloxacin, neither of which had any clinical impact.

A repeat sigmoidoscopy in October and November 2015 demonstrated stricture formation (Figure 1(d)–(g)). Histology confirmed a benign stricture with no evidence of spirochetes or CMV. Endoscopic dilation helped symptoms but repeat sigmoidoscopy in 2016 (Figure 1(h) and (i)) demonstrated re-stricturing despite negative microbiology. There has been no evidence of malignancy to date; however, recurrent dilation has been required to control symptoms and avoid complete stenosis. More invasive surgical options have been considered but were not acceptable to the patient whose frequency of dilatations has now reduced to twice yearly.

Discussion

The majority of cases of rectal LGV in Europe and North America have been identified amongst White men who have sex with men (MSM) presenting with proctitis.^1,2 It typically presents with purulent discharge, tenesmus and altered bowel habit. This may be associated with fever, malaise and weight loss. Endoscopic findings such as mucosal inflammation, ulceration and granulation tissue may be seen, ³ and may be similar to those caused by inflammatory bowel disease, rectal adenocarcinoma and lymphoma^3–6; hence, a high degree of clinical suspicion is warranted.

Screening is important as other sexually transmitted infections (including syphilis, gonorrhoea and herpes simplex virus ³ ) may also cause proctitis or may coexist with LGV, potentially having a synergistic affect.

Stricture formation has been very uncommon in >4000 MSM cases of LGV proctitis in the UK. Recurrent stricture formation in the absence of ongoing infection is even more unusual. Consensus on how best to manage these patients and the indications for surgery are not well established.

It is unclear as to why so few cases of LGV progress to stricture formation despite causing extensive inflammation, but delayed diagnosis is likely to be a factor. There may also be additional host and or pathogen factors involved. Prompt diagnosis and treatment may lead to a reduction in complications; avoid unnecessary treatment and the need for surgery. Gastroenterologists, colorectal surgeons and histopathologists need to be encouraged to consider LGV as a diagnosis in patients with proctocolitis. This gentleman had not been sexually active for a number of years prior to his diagnosis reflecting the importance of historical sexual behaviour and training in sexual history taking amongst clinicians in these fields.