Table 1.
Features of the patients’ diagnosis of Whipple’s disease after tumor necrosis factor alpha antagonist initiation
| Authors / country / language of publication | Age / sex | Underlying disease previous WD diagnosis / time with that diagnosis | TNF-α drugs and immunosuppressive treatment | Start of acute symptoms after TNF-α blockade | Symptoms after TNF-α blockade | Organ involvement | Histological diagnosisa | Result of PCR for Tropheryma whipplei | Treatment for WD | Outcome |
|---|---|---|---|---|---|---|---|---|---|---|
| Kneitz et al. (2005) [4] / Germany / English | 34y / M | SD | Methotrexate + infliximab (2w) | 2w | Weight loss, erythema nodosum, diarrhea, lymph node enlargement, and a sigmoidovesical fistula | Skin, gastroduodenitis and sigmoidovesical | Positive in fistula and lymph nodes | Positive in lymph node, small bowel and sigmoidovesical fistula | SXT (12m) | Resolved within 1 year |
| Kremer et al. (2008) [7] / Germany / German | 47y / M | SA / 4y | Leflunomide + adalimumab | Fever, weight loss, and severe arthralgia | Positive in duodenal tissue | Positive in duodenal tissue | SXT | |||
| Spoerl et al. (2009) [5] / Switzerland / English | 64y / M | SA / 5 + 3y | Etanercept | 4m | Lethargy, night sweats, weight loss | Gastrointestinal and vertebral | Positive in duodenal tissue and vertebral tissue | Positive in gastric and vertebral tissue | Ceftriaxone (2w), then, SXT (2y), (12m) then doxycycline + HC (18m) | No relapses after 34-month follow-up |
| Ahmadi-Simab et al. (2009) [8] / Germany / German | 33y / F | AS / 8y | Etanercept | 12m | Diarrhea, abdominal pain and weight loss | Negative in duodenal tissue | Positive in duodenal biopsy | Ceftriaxone (2w) after SXT | ||
| Hoppé et al. (2010) [6] / France / English | 67y / F | SA / 7y | Infliximab (4m) | 4m | Chest pain, dyspnea, polyarthritis | Positive in duodenal tissue | Positive in duodenal tissue. Negative in blood and saliva | Ceftriaxone (2w), then doxycycline (24m) | Resolved within 15d. No relapses after 71-month follow-up | |
| Hoppé et al. (2010) [6] / France / English | 40y / M | AS / 1y | Infliximab (18m) then etanercept (7m) then adalimumab (1m) | 26m | Diarrhea, weight loss, fever, arthralgia | Widespread ileocolitis, gastroduodenitis, meningitis | Positive in duodenal tissue | Positive in saliva, feces and cerebrospinal fluid. Negative in blood | Doxycycline, HC, and SXT (15d), then SXT was replaced by sulfasalazine 4g/day | Resolved within 2 months. No relapses after 17-month follow-up |
| Hoppé et al. (2010) [6] / France / English | 60y / M | AS / 8y | Etanercept (9m) | 9m | Fever, night sweats, polyarthritis, chest pain | Pericarditis, mediastinal and abdominal lymphadenopathy, duodenitis | Negative in duodenal tissue | Positive in the duodenal sample, saliva, feces, and lymph nodes | Doxycycline + HC (15m) | Resolved within 7d. No relapses after 30-month follow-up |
| Hoppé et al. (2010) [6] / France / English | 47y / M | RA / 17y | Infliximab (36m), then etanercept (42m), then adalimumab (6m), then rituximab, abatacept then infliximab (1m) | 85m | Fever, night sweats, weight loss, polyarthritis, radio carpal arthropathy. transient diplopia, myalgia, subacute depression | Pericarditis, abdominal lymphadenopathy, duodenitis | Positive PCR assay in the duodenal sample, blood, saliva, and feces. Negative PCR assay in cerebrospinal fluid | Doxycycline + HC (UT) | Resolved within 2m No relapses after 15-month follow-up | |
| Hoppé et al. (2010) [6] / France / English | 38y / M | AS / 9y | Etanercept (2m) | 9m | Diarrhea, abdominal pain. weight loss, blurred vision | Hemorrhagic gastroduodenitis, hemorrhagic colitis, mediastinal and cervical lymphadenopathy, splenomegaly, meningitis | Positive in duodenum and colon | Positive PCR assay in blood, saliva, cerebrospinal fluid, and feces | Doxycycline 200mg/day. HC 600mg/day. (UT) | Resolved within 3w. No relapses after 13-month follow-up |
| Hmamouchi et al. (2010) [9] / France / English | 35y / M | AS / 4y | Etanercept | 4m | Gingival nodule, purpura, abdominal pain diarrhea, fatigue, weight loss | Gingiva and colitis | Positive in colon tissue | Positive in blood, stool, and cerebrospinal fluid | SXT (NR) | Resolved within 3 months |
| Daïen et al. (2010) [10] / France / English | 70y / M | SA / 27y | Etanercept (18m) | NR | Fever and dyspnea | Endocarditis of aortic valve | Negative in duodenal tissue and, aortic valve | Positive in aortic valve | Doxycycline HC + SXT (18m) | Resolved. No relapses after 21-month follow-up |
| Gaddy et al., (2012) [12] / USA / English | 46y / M | AS / 10y | Prednisone + MTX + infliximab (24m), then adalimumab | 24m | Fever, migratory arthritis, weight loss, diarrhea | Gastroenteritis | Positive in duodenal tissue | Positive in blood and duodenal tissue | Ceftriaxone (x2w) after SXT | NR |
| Sparsa et al. (2013) [11] / France / French | 53y / M | SA | Etanercept (3m), then adalimumab (6m) | 9m | Polyarthralgia peripheric, arthritis metarsophalangeal | Positive in duodenal tissue biopsy | Positive in saliva and duodenal tissue | Doxycycline + HC (18m) | Resolved within 3 weeks. No relapses after 18-month follow-up | |
| Sparsa et al. (2013) [11] / France / French | 42y / M | SA / 2m | Adalimumab (9m) and then etanercept (4m) | 13m | Polyarthralgia peripheric | Positive in duodenal and gastric tissue | Positive in saliva and duodenal tissue | Doxycycline + HC and then doxycycline alone | Resolved within 3 weeks. No relapses after 17- month follow-up | |
| Present report | 58y / M | Psoriatic spondyloarthropathy/ 14y | Adalimumab (4m), then etanercept (8m), infliximab (2m), tocilizumab (21m), golimumab (1m) | 36m | Abdominal pain, diarrhea and weight loss | Duodenitis and abdominal lymph nodes | Positive in duodenal tissue | Not done | Ceftriaxone (14d), then SXT during (UT) | Resolved within 3 weeks. No relapses after 9-month follow-up. |
| Present report | 78y / M | RA / 19y | Infliximab (6m), etanercept (6m), rituximab (3m), leflunomide (24m) | 24m | Abdominal pain, diarrhea and weight loss | Duodenitis, and pericardial effusion | Positive in duodenal tissue | Positive in duodenal biopsy. Negative in cerebrospinal fluid | Ceftriaxone (14d), then SXT for (12m) then doxycycline + HC | Resolved within 3 weeks. No relapses after 18-month follow-up |
(UT) Patient still under treatment
TNF-α tumor necrosis factor alpha, WD Whipple’s disease, PCR polymerase chain reaction, y year, SD Still’s disease, w week, SXT cotrimoxazole, m month, SA spondyloarthropathy (seronegative chronic polyarthritis), HC hydroxychlorochine, AS ankylosis spondylitis, RA rheumatoid arthritis, NR not reported, MTX methotrexate
aHistological diagnosis = intracellular bacilli on periodic acid–Schiff stain