{
  "abstract": "Introduction Pulmonary hypertension (PH) is a rare pathophysiological disorder associated with many other medical conditions. Diagnosis of PH can be particularly complex when these associated conditions include rare infections such as Whipple’s disease. Here, we present the case of a woman initially diagnosed with sarcoidosis, who later developed severe PH and was subsequently found to have Whipple’s disease. 1 2 Case A 43-year-old woman presented with anaemia and generalised lymphadenopathy. A lymph node biopsy revealed non-necrotising granulomatous inflammation which led to an initial diagnosis of sarcoidosis. Treatment with corticosteroids was commenced. During routine cardiac evaluation, echocardiography showed severe pulmonary hypertension (pulmonary artery systolic pressure (PASP) 80 mmHg with right ventricular dysfunction). Right heart catheterisation confirmed pre-capillary PH with a mean pulmonary artery pressure (mPAP) of 29 mmHg and normal wedge pressure. She was diagnosed as having PH associated with sarcoidosis and started on sildenafil. However, the patient developed persistent diarrhoea, a known side effect of sildenafil, which prompted a switch to tadalafil. Despite this, gastrointestinal symptoms persisted. Extensive investigations, including endoscopy and duodenal biopsy, unexpectedly led to the diagnosis of Whipple’s disease. She was treated with intravenous ceftriaxone for two weeks, followed by 12 months of co-trimoxazole therapy. Following the initiation of antibiotic therapy, the patient’s gastrointestinal symptoms improved significantly. Her diarrhoea resolved, and weight gain was noted. After 12 months of co-trimoxazole, a repeat echocardiogram demonstrated a reduction in PASP to 42 mmHg, with normal function of the right ventricle. This clinical improvement supported the hypothesis that the resolution of PH was, at least partly, related to the effective treatment of Whipple’s disease. 3 4 Discussion Whipple’s disease is a rare systemic infection caused by Tropheryma whipplei, with the gastrointestinal system being most affected, patients presenting with diarrhoea, malabsorption, and weight loss. Though Whipple’s disease is primarily a gastrointestinal disease, it can involve multiple organs, including the cardiovascular system. Whipple’s disease has been rarely reported as a cause of PH.5 The exact mechanisms linking Whipple’s disease to PH remain unclear. One theory posits that the accumulation of periodic acid-Schiff (PAS)-positive macrophages within the pulmonary vasculature may increase pulmonary vascular resistance, thus contributing to the development of PH (figure 1). Moreover, the reversibility of PH upon appropriate antibiotic treatment lends support to a direct association between the two conditions.6 7 The diagnosis of PH in this patient was initially confounded by the suspicion of sarcoidosis, another cause of PH, and the side effects of sildenafil. This case demonstrates the diagnostic complexity of PH complicating many systemic diseases and the importance of considering rare infectious causes such as Whipple’s disease when gastrointestinal symptoms remain unexplained. A further learning point is that PH associated with Whipple’s disease can improve with antibiotic therapy, reinforcing the need for early correct diagnosis and treatment.Abstract 7-043 Figure 1Periodic Acid-Schiff (PAS) positive staining in macrophagesReferences McGinnis SW, Simms JR. Pulmonary hypertension associated with sarcoidosis: Clinical implications and management strategies. Chest. 2018;153(3):630–639. doi: 10.1016/j.chest.2017.11.050.Smoot JM, Alvarado LR. The role of pulmonary hypertension in systemic diseases: Review of mechanisms and management. J Clin Med. 2020;9(3):775. doi: 10.3390/jcm9030775.Dostal CL, Kollef MH. Tadalafil and ambrisentan for the treatment of pulmonary hypertension. Am J Respir Crit Care Med. 2018;198(9):1157–1168. doi: 10.1164/rccm.201804-0715OC.Soler M, González J, Díaz M, et al. Resolution of pulmonary hypertension with antibiotic therapy in Whipple's disease: A case series. Eur Respir J. 2017;50(6):1700876. doi: 10.1183/13993003.00876-2017.Desnues B, Al Moussawi K, Fenollar F. New insights into Whipple's disease and Tropheryma whipplei infections. Microbes Infect. 2010 Dec;12(14–15):1102–10. doi: 10.1016/j.micinf.2010.08.001.Villanueva R, et al. Pulmonary involvement in Whipple’s disease: Case report and review of the literature. Chest. 2015;148(4):968–974. doi: 10.1378/chest.15-0866.Cacoub P, et al. The pathophysiology of pulmonary hypertension in chronic inflammatory diseases. Curr Opin Pulm Med. 2017;23(4):305–312. doi: 10.1097/MCP.0000000000000389.",
  "authors": [
    {
      "affiliations": [
        "Royal Brompton Hospital, Guys and St Thomas NHS Foundation Trust",
        "Imperial College Healthcare NHS Trustzz"
      ],
      "name": "Syeda Anum Zahra"
    },
    {
      "affiliations": [
        "Royal Brompton Hospital, Guys and St Thomas NHS Foundation Trust"
      ],
      "name": "Colm McCabe"
    },
    {
      "affiliations": [
        "Royal Brompton Hospital, Guys and St Thomas NHS Foundation Trust"
      ],
      "name": "Laura Price"
    },
    {
      "affiliations": [
        "Royal Brompton Hospital, Guys and St Thomas NHS Foundation Trust"
      ],
      "name": "Michael Brown"
    },
    {
      "affiliations": [
        "Royal Brompton Hospital, Guys and St Thomas NHS Foundation Trust"
      ],
      "name": "Heba Nashat"
    },
    {
      "affiliations": [
        "Royal Brompton Hospital, Guys and St Thomas NHS Foundation Trust"
      ],
      "name": "Stephen John Wort"
    }
  ],
  "title": "7-043 Pulmonary hypertension and whipple’s disease: a diagnostic challenge",
  "uid": "174327ef-6000-5d39-b987-e030375bd059"
}