Background Malakoplakia is a rare condition characterized by inflammatory masses with specific histological characteristics. These soft tissue masses can mimic tumors and tend to develop in association with chronic or recurrent infections, typically of the urinary tract. A specific defect in innate immunity has been described. In the absence of randomized controlled trials, management is based on an understanding of the biology and on case reports. Case presentation Here we describe a case of presacral malakoplakia in a British Indian woman in her late 30s, presenting with complex unilateral foot drop. Four years earlier, she had suffered a protracted episode of intrapelvic sepsis following a caesarean delivery. Resection of her presacral soft tissue mass was not possible. She received empiric antibiotics, a cholinergic agonist, and ascorbic acid. She responded well to medical management both when first treated and following a recurrence of symptoms after completing an initial 8 months of therapy. Whole exome sequencing of the patient and her parents was undertaken but no clear causal variant was identified. Conclusions Malakoplakia is uncommon but the diagnosis should be considered where soft tissue masses develop at the site of chronic or recurrent infections. Obtaining tissue for histological examination is key to making the diagnosis. This case suggests that surgical resection is not always needed to achieve a good clinical and radiological outcome.
20.4%) patients had complicated disease behaviour compared to 46 (34.9%) at follow up (p=0.0018). 83 (62.9%) patients had a 'pan-enteric' phenotype at diagnosis, however only 55 (66.3%) retained this phenotype at follow-up (p=0.0002). Disease extension was noted in 18.9% of patients and involution in 35.6% of patients, with upper GI disease noted in only 15.9% of patients at follow-up (p=0.0001). There was a high exposure to both thiopurines (91.7%) and biologics (63.6%), with a median time to starting treatment of 0 (IQR 0-1) and 5 (IQR 2-7) years for thiopurines and anti-TNF therapy respectively. The rate of exposure to biological therapy was similar in patients with disease involution (32/47, 68.1%) and disease extension (21/25, 84%). The cumulative probability (95% CI) of surgery was 0.05 (0.02, 0.11) at 1 year, 0.17 (0.11, 0.24) at 3 years and 0.22 (0.15, 0.30) at 5 years respectively. Overall, 56 (42.4%) patients had surgery at the end of follow-up. Neither disease location nor behaviour were associated with need for surgery. Conclusions Changes in both disease location and behaviour were seen in our PCD cohort as they progressed to adult life. A significant proportion had disease involution, likely related to a high rate of exposure to biological therapy.
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