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Background Recently, Tropheryma whipplei has been suggested as one of the causative agents of diarrhea among children worldwide. Limited data is available on the prevalence of T. whipplei among children with diarrhea in most countries such as Iran. This study was conducted to evaluate the prevalence of T. whipplei in children with acute diarrhea in Iran. Methods In this study, the stool samples were collected from 130 children under 10 years old with acute diarrhea from children's hospitals in Tehran city. Genomic DNA was extracted from stool samples and was tested for the presence of DNA of T. whipplei using the SYBR Green Real-time PCR method. Positive T. whipplei samples were finally confirmed by PCR Product sequencing. Results The mean age of participants was 32.5 months, and 54.6% of children were female. Using the SYBR Green Real-time PCR, 9.23% (12/130) of samples were positive for T. whipplei, which were confirmed by sequencing. 66.67% of positive cases were males. The duration of diarrhea in infected children with T. whipplei (83.3%) was significantly longer (OR: 5.93, 95% CI 1.24–28.22) compared to children with negative results (45.8%). Other demographic factors and clinical signs had not a statistically significant relationship with T. whipplei infection. Conclusions In this study, T. whipplei was detected in stool samples of children with acute diarrhea. The results indicated that T. whipplei could be associated with childhood diarrhea in Iran. The health care system and physicians should be aware of the presence of T. whipplei infection in Iran, especially in childhood diarrhea.

Neurologic manifestations occur in 20%–40% of patients with Whipple disease, a rare infectious disorder caused by the bacterium Tropheryma whipplei.

Background Whipple’s disease is a clinically relevant multi-system disorder that is often undiagnosed given its elusive nature. We present an atypical case of Whipple’s disease involving pan-valvular endocarditis and constrictive pericarditis, requiring cardiac intervention. A literature review was also performed assessing the prevalence of atypical cases of Whipple’s disease. Case presentation A previously healthy 56-year-old male presented with a four-year history of congestive heart failure with weight loss and fatigue. Notably, he had absent gastrointestinal symptoms. He went on to develop pan-valvular endocarditis and constrictive pericarditis requiring urgent cardiac surgery. A clinical diagnosis of Whipple’s disease was suspected, prompting duodenal biopsy sampling which was unremarkable, Subsequently, Tropheryma whipplei was identified by 16S rDNA PCR on the cardiac valvular tissue. He underwent prolonged antibiotic therapy with recovery of symptoms. Conclusions Our study reports the first known case of Whipple’s disease involving pan-valvular endocarditis and constrictive pericarditis. A literature review also highlights this presentation of atypical Whipple’s with limited gastrointestinal manifestations. Duodenal involvement was limited and the gold standard of biopsy was not contributory. We also highlight the Canadian epidemiology of the disease from 2012 to 2016 with an approximate 4% prevalence rate amongst submitted samples. Routine investigations for Whipple’s disease, including duodenal biopsy, in this case may have missed the diagnosis. A high degree of suspicion was critical for diagnosis of unusual manifestations of Whipple’s disease.

Background Whipple’s disease is caused by Tropheryma whipplei and causes a self-limiting gastrointestinal infection. The majority of the population is an asymptomatic carrier, however, in some patients, it causes an invasive infection with for example arthritis, endocarditis, or involvement of the eyes. Case summary This case describes a man with long-lasting complaints of progressive dyspnoea caused by heart failure due to total destruction of the aortic and mitral valve as a result of T. whipplei endocarditis, diagnosed with serum polymerase chain reaction. The patient was treated with ceftriaxone and prolonged co-trimoxazole therapy and surgical replacement of the aortic and mitral valve. He was discharged to a rehabilitation centre. Discussion Tropheryma whipplei is one of the possible microorganisms classified as causing blood culture-negative endocarditis, with predominantly afebrile patients that do not fulfil the Dukes criteria, which makes it difficult to diagnose. Polymerase chain reaction is the cornerstone of the diagnosis. It requires long-term antibiotic treatment up to 12 months. It is recommended by the European Society of Cardiology to discuss treatment in an Endocarditis Team because Whipple’s endocarditis has only rarely been described in the literature previously. Whipple’s endocarditis has high mortality and relapse rates.

We report 2 cases of Whipple disease (WD), previously diagnosed as seronegative polyarthritis and treated for several years with immunosuppressive agents, accordingly. Both cases had been treated over years with cDMARDs and bDMARDs. The first patient was a 48-year-old male, who developed a life-threatening disease characterized by fever, significant weight loss, and bloody diarrhoea, supported with RBC transfusions. The second patient was a 55-year-old man, presenting with arthritis, fever, serositis, lymphadenopathy, thoracic rash, and systemic inflammation; at the beginning he was diagnosed as adult onset Still’s disease. He was treated with steroids and antitumour necrosis factor agents, but showed no improvement. Both patients were eventually treated with antimicrobial therapy for WD with dramatic improvement and no clinical relapse in 6 months. This paper reviews the literature on WD mimicking chronic inflammatory arthritis. WD may lead to chronic seronegative arthritis that might often be misrecognized. Importantly, patients treated with bDMARDs and glucocorticoids might develop a life-threatening disease. Therefore, WD should be suspected and excluded in patients showing resistance or frequent recurrence of chronic arthritis, if seronegative, under treatment with bDMARDs, especially in the presence of new, unexpected sign and/or symptoms.

Systemic lupus erythematosus (SLE) is a multisystemic autoimmune disease that can affect major organs possibly leading to life-threatening complications and appears with heterogeneous clinical picture. SLE could present with broad spectrum of clinical and laboratory features that can resemble those of other diseases, such as hemopoietic malignancies, infections, or immune-mediated disorders. Its complexity and protean features overlap with many other diseases, hindering the differential diagnosis. Rarely, true overlap with other diseases may occur. Herein, we report a case series of two patients affected by infectious diseases, namely visceral leishmaniasis and Whipple’s disease (WD), intertwined with clinical or serological features of SLE. In both cases, several confounding factors have led to a delay in the diagnosis. Moreover, we first describe the persistent elevation of autoantibodies and a monoclonal gammopathy in a patient with WD. Awareness of unusual presentations of infections or other rare disorders, which may be encountered in clinical practice when taking care of SLE patients, is essential for timely diagnosis and treatment of potentially lethal diseases.

Whipple's disease is a rare, multisystem infection caused by the Gram-positive Tropheryma whippelii organism. In addition to neurological and rheumatological manifestations, this disease can result in significant gastrointestinal symptoms such as malabsorption, diarrhea, and weight loss. Given the diagnostic challenge and rare occurrence, a high index of suspicion is critical to prevent morbidity and mortality from this otherwise highly infectious disease transmitted via the fecal-oral route. We present a very rare but near-fatal case of hypovolemic shock secondary to protein-losing enteropathy and gastrointestinal bleeding from small bowel T. whippelii infection. Furthermore, the epidemiology, clinical presentation, diagnosis, and management of Whipple's disease is reviewed.

Histiocytic proliferations are heterogeneous lesions with distinct pathogenic and clinical-pathological features. While many of these conditions are nonspecific immune responses to variable causes, a minority of them is associated with specific etiological factors and unique clinical-pathological pictures. By presenting a rare case of Whipple adenopathy, we address the peculiar histological features of this condition and the differential diagnosis of nodal histiocytosis in general.

International audience; Tropheryma whipplei is the agent of Whipple’s disease, a rare systemic disease characterized by macrophage infiltration of the intestinal mucosa. The disease first manifests as arthralgia and/or arthropathy that usually precede the diagnosis by years, and which may push clinicians to prescribe Tumor necrosis factor inhibitors (TNFI) to treat unexplained arthralgia. However, such therapies have been associated with exacerbation of subclinical undiagnosed Whipple’s disease. The objective of this study was to delineate the biological basis of disease exacerbation. We found that etanercept, adalimumab or certolizumab treatment of monocyte-derived macrophages from healthy subjects significantly increased bacterial replication in vitro without affecting uptake. Interestingly, this effect was associated with macrophage repolarization and increased rate of apoptosis. Further analysis revealed that in patients for whom Whipple’s disease diagnosis was made while under TNFI therapy, apoptosis was increased in duodenal tissue specimens as compared with control Whipple’s disease patients who never received TNFI prior diagnosis. In addition, IFN-γ expression was increased in duodenal biopsy specimen and circulating levels of IFN-γ were higher in patients for whom Whipple’s disease diagnosis was made while under TNFI therapy. Taken together, our findings establish that TNFI aggravate/exacerbate latent or subclinical undiagnosed Whipple’s disease by promoting a strong inflammatory response and apoptosis and confirm that patients may be screened for T. whipplei prior to introduction of TNFI therapy.

Whipple's disease is a rare infectious disease caused by the bacterium Tropheryma whipplei. The prevalence of Whipple's disease is 3/1,000,000 in Western populations. It most often causes a malabsorption disorder with weight loss and diarrhea as common presenting symptoms. In one-third of patients, however, there are no gastrointestinal symptoms at presentation; patients instead report a wide variety of non-specific extraintestinal complaints, potentially involving every organ system. We report a case of a 37-year-old man who presented with a 3-month history of non-bloody diarrhea and 15-pound weight loss. He was ultimately diagnosed with biopsy-confirmed Whipple's disease. Despite its rarity, Whipple's disease remains an important clinical entity and should be included on the differential diagnosis for selected patients presenting with an array of non-specific symptoms.

Malabsorptive bariatric techniques are associated with nutritional deficiencies. However, when patients do not respond to supplemental intensive treatments they should be closely followed because they can hide other pathological conditions. We present the case of a 47-year-old man with morbid obesity (body mass index [BMI]: 48 kg/m2) who underwent bariatric surgery. In 2016, he presented severe pneumonia and hospitalization at the Intensive Unit Care was required. After this episode, his nutritional state impaired, presenting 6-7 diarrhea/steatorrhea events per-day and requiring several hospitalizations due to the persistence of severe hypoproteinemia. He was given parenteral high-protein associated with low-fat oral diet. He presented a temporary biochemical improvement, but the hypoproteinemia recurred. Finally, tests revealed the presence of Tropheryma whipplei as protein-losing enteropathy. Whipple's disease (WD) is a rare cause of diarrhea and malnutrition, and these symptoms can be confused with the postoperative status of malabsorptive bariatric techniques. WD requires early diagnosis with prolonged antibiotic treatment to avoid severe complications. Resumen Las técnicas bariátricas malabsortivas suelen asociarse a deficiencias nutricionales. Sin embargo, cuando los pacientes no responden a tratamientos intensivos suplementarios, deben valorarse otras condiciones patológicas. Presentamos el caso de un hombre de 47 años, obeso mórbido (índice de masa corporal [IMC]: 48 kg/m2) sometido a cirugía bariátrica, que dos años más tarde presentó neumonía severa, por lo que requirió ingreso en la Unidad de Cuidados Intensivos. Posteriormente, el estado nutricional se deterioró, presentando 6-7 episodios de diarrea-esteatorrea/día y requiriendo varias hospitalizaciones por hipoproteinemia severa. Recibió infusión parenteral rica en proteínas asociada con una dieta baja en grasas y presentó mejoría analítica temporal. Finalmente, las pruebas revelaron la presencia de Tropheryma whipplei, una bacteria que genera enteropatía pierde-proteínas. La enfermedad de Whipple (EW) es una causa poco común de diarrea y malnutrición. Estos síntomas pueden confundirse con el posoperatorio de técnicas bariátricas malabsortivas. La EW requiere un diagnóstico precoz con un tratamiento antibiótico prolongado para evitar complicaciones graves.

Background/Aims A 45-year-old male patient presented in 2015 with a six-month history of relapsing and remitting polyarthralgia. Hand X-rays appeared normal. Serology showed mildly elevated inflammatory markers. Autoimmune profile including anti-CCP antibody, rheumatoid factor and ANA was negative. His initial diagnosis was palindromic rheumatism. He was under watchful waiting in rheumatology clinic having declined a trial of hydroxychloroquine, when in 2018 he developed severe epigastric pain. Over the subsequent 18 months he was noted to have dramatic weight loss, fatigue and drenching night sweats. Methods Investigations showed microcytic anaemia with elevated inflammatory markers (Hb 98 g/L, CRP 161 mg/L, ESR 68 mm/hr). Serum ACE, bone profile, thyroid function and urate levels were normal. Chest X-ray was unremarkable. HIV and hepatitis screening was negative. Endoscopy with jejunal biopsy was performed, with mild gastritis only on histopathology and normal D2 biopsies. He was found to be H pylori positive, and notably felt his B-symptoms much improved with triple antibiotic and PPI eradication therapy. CT abdomen demonstrated widespread mesenteric lymphadenopathy. Para-aortic lymph node biopsy showed non-necrotising granulomata suggestive of either sarcoidosis or an infective etiology such as tuberculosis (TB). Given the clinical picture, the patient was commenced on high dose oral prednisolone and methotrexate for suspected sarcoidosis. Results The patient made some clinical improvement, particularly with regards to arthralgia, however his B-symptoms returned with any reduction in steroid dose. Serology showed worsening anaemia with iron and folate deficiency, and increasing inflammatory markers. We therefore decided to perform a PET CT and refer to Haematology for consideration of a lymphoproliferative disease. PET CT demonstrated lymphadenopathy without avid uptake. A second lymph node biopsy was performed which showed florid histiocytic infiltration within which there were numerous PAS positive particles consistent with Whipple's disease. This was confirmed as tropheryma whipplei on PCR. Whipple's disease is a rare systemic infectious disease causing arthralgia, diarrhoea, abdominal pain and weight loss. Treatment consists of antibiotic therapy. On further questioning, the patient had grown up on a farm. There is a known association with Whipple's disease in the agricultural community as it is a soil-borne organism. Conclusion Our patient has made an excellent recovery. He remains under the care of our rheumatology team and the London School of Tropical Medicine and Hygiene. Treatment plan is 12 months of combined therapy with doxycycline and hydroxychloroquine. He underwent a lumbar puncture to rule out meningeal Whipple disease. We have also commenced sulfasalazine for persistent arthritis, which we feel could be a reactive phenomenon. Our key learning points from this case were to use a stepwise approach to diagnosis, involve relevant specialty teams and that it in complex cases it is useful to go back to the history. Disclosure F. Baldeweg: None. A. Nuttall: None. D. Arul: None. A. Childerhouse: None.

Most patients with Whipple’s disease have rheumatic symptoms. The aim of our prospective, questionnaire-based, non-interventional clinical study was to assess whether these symptoms are useful in guiding the differential diagnosis to the rheumatic disorders. Forty patients with Whipple’s disease, followed by 20 patients for validation and 30 patients with rheumatoid-, 21 with psoriatic-, 15 with palindromic- and 25 with axial spondyloarthritis were recruited for the present investigation. Patients with Whipple’s disease and patients with rheumatic disorders were asked to record rheumatic symptoms on pseudonymized questionnaires. The data obtained were subjected to multiple logistic regression analysis. Episodic pain with rapid onset, springing from joint to joint was most common in patients with palindromic arthritis and second most common and somewhat less conspicuous in Whipple’s disease. Continuous pain in the same joints predominated in patients with rheumatoid-, psoriatic-, and axial spondyloarthritis. Multiple logistic equations resulted in a predicted probability for the diagnosis of Whipple’s disease of 43.4 ± 0.19% (M ± SD) versus a significantly lower probability of 23.8 ± 0.19% (M ± SD) in the aggregate of patients with rheumatic disorders. Mean area under the curve (AUC) ± SD was 0.781 ± 0.044, 95% CI 0.695–0.867, asymptotic significance p

Myorhythmia is a hyperkinetic movement disorder characterized by slow, repetitive, rhythmic, cranial and limb contractions, typically disappearing during sleep. Infectious, autoimmune or vascular lesions involving brainstem or diencephalic structures are the most common etiologies.1 Anti-IgLON5 disease was originally reported as a progressive neurological syndrome characterized by a preeminent sleep disorder, variably associated with bulbar dysfunctions, gait instability, oculomotor abnormalities and cognitive decline.2, 3 Since initial descriptions, several hyperkinetic movement disorders have been described, including oro-facial myorhythmia in few cases.4, 5 We describe a case of anti-IgLON5 disease-related myorhythmia involving both facial and limb muscles, persisting during physiological and pathological sleep.

Whipple disease (WD) is a rare bacterial infectious disease that is classically characterized by years of arthralgia, followed by malabsorption, diarrhea, and weight loss. However, WD may manifest in virtually any organ system, and patients with WD rarely develop subcutaneous erythema nodosum-like lesions. We report a case of a 51-year-old man diagnosed with WD who subsequently developed widely distributed erythematous subcutaneous nodules after 5 months of antibiotic therapy.

To describe posterior ocular involvement features of Whipple's disease (WD) in a patient with no gastrointestinal symptoms.Retrospective case report.A 53-year-old man with a 2-year history of seronegative arthritis presented with bilateral intraocular inflammation, optic disc edema, and cystoid macular edema (CME) in the left eye. A diagnosis of noninfectious uveitis was made and oral prednisolone was started. Despite initial improvement, after 6 weeks, CME was found in both eyes. Because of the initial response, the anti-tumor necrosis factor agent Adalimumab was started. Twelve weeks after initiation of adalimumab, fundus examination revealed widespread dot-blot retinal hemorrhages and multifocal chorioretinal lesions at the posterior pole and mid-periphery. The chorioretinal lesions appeared as hyperreflective drusen-like deposits located in the sub-retinal pigment epithelium (RPE) space on the tomographic scan. WD was considered and confirmed by polymerase chain reaction test and duodenal biopsy.Posterior ocular involvement in WD may present with a wide clinical spectrum including intraocular inflammation and unique features of sub-RPE deposits, widespread retinal hemorrhages, and optic disc edema.

Background Whipple’s disease is a chronic infectious disease caused by the Gram-positive bacterium Tropheryma whipplei (TW), which not only affects the gastrointestinal tract and causes malabsorption of nutrients, but several other systems, such as the cardiovascular system, central nervous system, the joints, and the vascular system, can also be simultaneously involved. The aim of this report was to be able to alert the clinician to severe pneumonia caused by TW combined with Candida sp. Case presentation The case study was conducted on patients in September and November 2019. After routine examination and treatment, the results were not satisfactory. A bronchoalveolar lavage (BAL) using metagenomics next-generation sequencing was conducted on two adults who presented with fever, cough, and progressive dyspnea and who had no history of gastrointestinal symptoms, immunodeficiency diseases, or use of immunosuppressive agents. TW and Candida sp. were detected in in BAL. Conclusions This is a report of life-threatening pneumonia caused by TW combined with Candida sp. in a Chinese population.

Tropheryma whipplei is a bacterium that causes a rare infection called Whipple's disease and can cause devastating effects if left untreated. It is important to recognize that patients with this infection may present with atypical symptoms and are often apyrexial with normal inflammatory markers. Moreover, routine blood cultures often do not isolate these bacteria in conventional growth media. Therefore, it requires a high level of clinical suspicion to make this diagnosis. Here, we present two cases of Tropheryma whipplei aortic valve endocarditis, with atypical presentation and similar unusual but striking echocardiographic images.

Whipple's disease (WD) can mimic chronic inflammatory rheumatism leading to incorrect prescription of tumor necrosis factor inhibitors (TNFI). Several complicated cases of WD have been reported during TNFI treatment which is strongly suspected to modify the host-pathogen relationship. Tropheryma whipplei asymptomatic carriage is high in the general population, making the diagnosis of WD more difficult face to unexplained arthritis.We report three observations that illustrate situations for which the detection of T. whipplei might be valuable to investigate the differential diagnosis of inflammatory rheumatism.The decision to check for T. whipplei infection should rely on individual clinical assessment. It should be considered in the absence of clinical response or in case of worsening of an inflammatory rheumatism under TNFI treatment, especially in front of atypical features. A systematic screening for T. whipplei before anti-TNF treatment seems unjustified since asymptomatic carriers are frequent.

Background Whipple's disease is a chronic infectious disease that primarily affects the small intestine, but several organs can simultaneously be involved. The disease is caused by a gram-positive bacterium called Tropheryma whipplei. The disease is difficult to suspect because it is rare with unspecific and long-term symptoms; it can be lethal if not properly treated. Case presentation We here present three patients who presented with a plethora of symptoms, mainly long-standing seronegative arthritis and gastrointestinal symptoms in the form of diarrhea with blood, weight loss, fever, and lymphadenopathy. They were after extensive investigations diagnosed with Whipple's disease, in two of them as long as 8 years after the first occurrence of joint manifestations. The diagnosis was made by PCR targeting the T. whipplei 16S rRNA gene from small bowel specimen in all three patients, and, besides from histopathologic findings from the duodenum and distal ileum in one and mesenteric lymph nodes in another patient. Conclusions This report aims to raise awareness of a very rare disease that presents with a combination of symptoms mimicking other and significantly more common diseases.

Tropheryma whipplei, is an actinobacterium that causes different infections in humans, including Whipple’s disease. The bacterium infects and replicates in macrophages, leading to a Th2-biased immune response. Previous studies have shown that T. whipplei harbors complex surface glycoproteins with evidence of sialylation. However, the exact contribution of these glycoproteins for infection and survival remains obscure. To address this, we characterized the bacterial glycoprofile and evaluated the involvement of human β-galactoside-binding lectins, Galectin-1 (Gal-1) and Galectin-3 (Gal-3) which are highly expressed by macrophages as receptors for bacterial glycans. Tropheryma whipplei glycoproteins harbor different sugars including glucose, mannose, fucose, β-galactose and sialic acid. Mass spectrometry identification revealed that these glycoproteins were membrane- and virulence-associated glycoproteins. Most of these glycoproteins are highly sialylated and N-glycosylated while some of them are rich in poly-N-acetyllactosamine (Poly-LAcNAc) and bind Gal-1 and Gal-3. In vitro, T. whipplei modulates the expression and cellular distribution of Gal-1 and Gal-3. Although both galectins promote T. whipplei infection by enhancing bacterial cell entry, only Gal-3 is required for optimal bacterial uptake. Finally, we found that serum levels of Gal-1 and Gal-3 were altered in patients with T. whipplei infections as compared to healthy individuals, suggesting that galectins are also involved in vivo. Among T. whipplei membrane-associated proteins, poly-LacNAc rich-glycoproteins promote infection through interaction with galectins. T. whipplei modulates the expression of Gal-1 and Gal-3 both in vitro and in vivo. Drugs interfering with galectin–glycan interactions may provide new avenues for the treatment and diagnosis of T. whipplei infections.

Whipple disease (WD) is a rare infectious systemic disease. Rheumatologists are at the frontline of WD diagnosis due to the early rheumatological manifestations. An early diagnosis is crucial, as usual anti-rheumatic drugs, especially TNF inhibitors, may worsen the disease course. We conducted a retrospective multicentre national study from January 2010 to April 2020 to better characterize the rheumatological features of WD. Classic WD (CWD) was defined by positive periodic acid-Schiff (PAS) staining of a small-bowel biopsy sample, and non-CWD (NCWD) was defined by negative PAS staining of a small-bowel biopsy sample but at least one positive Tropheryma whipplei (TW) polymerase chain reaction (PCR) for a digestive or extradigestive specimen. Sixty-eight patients were enrolled, including 11 CWD patients. Twenty patients (30%) received TNF inhibitors during the WD course, with inefficacy or symptom worsening. More digestive symptoms and systemic biological features were observed in CWD patients than in NCWD patients, but both patient groups had similar outcomes, especially concerning the response to antibiotics and relapse rate. Stool and saliva TW PCR sensitivity were both 100% for CWD and 75% for NCWD and 89% and 60% for small-bowel biopsy sample PCR, respectively. WD encountered in rheumatology units has many presentations, which might result from different pathophysiologies that are dependent on host immunity. Given the heterogeneous presentations and the presence of chronic carriage, multiple TW PCR tests on samples from specific rheumatological sites when possible should be performed, but samples from nonspecific digestive and extradigestive sites also have great value.

Whipple’s Disease (WD) was first described by George H. Whipple in 1907. Classic Whipple disease (CWD) is a systemic chronic infection by Tropheryma whipplei (T. whipplei) that can involve various organ systems such as gastrointestinal tract, joints, and central nervous system (CNS). Central nervous system Whipple’s disease (CNS- WD) can be a part of the classical WD, a recurrent phenomenon or an isolated disease. It encompasses a wide array of clinical manifestations depending on the anatomic region affected by the pathological process. Diagnosis is made by polymerase chain reaction testing. It is vital to implement an aggressive mode of treatment to reduce the chances of complications like cognitive impairment, or disease recurrence, even many years later.

Significance Whipple disease is a rare chronic, systemic bacterial infection that predominantly affects the small intestine but also other organs of the body. When left untreated, it can be not only vision threatening but also life threatening because of its central nervous system involvement. Therefore, early detection and treatment are important. Purpose We report a rare case of unilateral optic disc edema as a critical identifying sign of Whipple disease. Case report An asymptomatic 49-year-old African American man presented for an eye examination and was found to have optic nerve edema of the right eye. His best-corrected visual acuity was 20/20 in the right and left eye. He denied symptoms of diplopia, amaurosis fugax, or eye pain. His medical history was significant for HIV with no recent detectable viral load at the time of his eye examination. The patient denied any other infectious risk factors or changes in medical status. Extensive ophthalmic, neuroimaging, and laboratory investigations were completed as a comprehensive approach to rule out more common etiologies for unilateral optic disc edema. This initial workup yielded no identifying etiology, and the patient was monitored closely with frequent examinations with a retina specialist. Soon after his diagnosis of optic nerve edema, the patient developed new symptoms of chronic diarrhea, weight loss, and fatigue requiring hospitalization. Evaluations by internal medicine and gastroenterology, including serological testing, stool analysis, histological and microbiological analysis, esophagogastroduodenoscopy, and gastrointestinal biopsy, confirmed a diagnosis of Whipple disease that was successfully treated with oral antibiotics. Conclusions Whipple disease is a rare cause of infectious optic nerve edema that may present with other rheumatoid and gastrointestinal symptoms. A comprehensive medical approach for investigating unilateral optic nerve edema is paramount in diagnosing and treating Whipple disease.

Vasculitis can also present with GI or solid organ involvement. IgA and ANCA associated vasculitis are more likely to have GI involvement. A 56-year-old female was admitted to the ER due to nausea, vomiting, epigastric pain and fever. The patient had a medical history of acromegaly and chronic kidney disease of an undetermined etiology, elevated C-reactive protein and renal dysfunction. Abdominal-CT revealed duodenal parietal thickening and pancreatic head edema. On esophagogastroduodenoscopy (EGD), duodenal mucosa had a diffusely nodular aspect with ulcerated areas. The following differential diagnosis were made, infectious enteritis, Whipple disease, infiltrative disorder and GI vasculitis. After discussion between a multidisciplinary team of Gastroenterology and Nephrology, they decided to initiate oral glucocorticoids due to worsening of the renal function, which lead to the resolution of digestive symptoms and renal function stabilization. Myeloperoxidase antineutrophilic-cytoplasmic antibodies (MPO-ANCA) were subsequently positive and histology confirmed duodenal involvement by vasculitis. The patient was asymptomatic after 4-weeks, with endoscopic healing and renal function stabilization. GI involvement limited to the duodenum in the setting of ANCA-MPO vasculitis is a rare condition. Moreover, histopathologic confirmation of vasculitis in endoscopic biopsy samples is exceptional.

Whipple disease is an infection caused by the bacterium Tropheryma whipplei. Due to its unspecific clinical symptoms, it is difficult to diagnose and often remains undetected for a long time. The case of a patient who presented with acute intestinal symptoms to the authors' department is reported. The diagnosis of classic Whipple disease was established. The symptoms subsided under antibiotic therapy. Complications in the form of immune reconstitution inflammatory syndrome (IRIS) occurred, requiring immunosuppressive treatment.Der Morbus Whipple ist eine durch das Bakterium Tropheryma whipplei verursachte Infektion, deren Diagnosestellung durch eine unspezifische Beschwerdesymptomatik erschwert wird und die häufig lange unerkannt bleibt. Wir berichten über einen Patienten, der sich mit neu aufgetretenen intestinalen Beschwerden in unserer Abteilung vorstellte. Es konnte die Diagnose eines klassischen Morbus Whipple gestellt werden. Unter antibiotischer Therapie wurde eine Remission der Symptomatik erreicht. Komplizierend traten Beschwerden im Sinne eines Immunrekonstitutionssyndroms (IRIS) auf, die eine immunsuppressive Behandlung erforderten.

A 51-year-old man presented with watery diarrhea and 7-kg weight loss for six months. As computed tomography scans showed multiple mesenteric and retroperitoneal lymphadenopathy, 18F-fluoro-deoxyglucose positron emission tomography was performed, showing marked accumulation in the jejunum. Enteroscopy disclosed diffuse yellow-white swollen villi in the jejunum and biopsies revealed clubbed villi with marked periodic acid-Schiff-positive foamy macrophages. The electron microscopic examination showed numerous extracellular bacilli and bacillary forms of degradation in the macrophages. 16S rRNA PCR revealed Tropheryma whipplei in the mucosal specimens, making the diagnosis of Whipple’s disease. Therapy with ceftriaxone followed by long-term trimethoprim-sulfamethoxazole was effective. Whipple's disease is briefly discussed.

Whipples disease (WD) is a rare infectious disease with an annual incidence of 1-6:10.000.000. It is mostly found in middle aged, Caucasian males. The causative bacterial agent Tropheryma whipplei (TW) is common, but only few infected with TW develop actual WD pointing to host factors as essential. We present a case story of classic WD seen in a 48-year-old man presumed to have sarcoidosis and therefore treated with prednisolone which caused WD to flare up and ultimately unmask the disease.

A 59-year-old female patient was diagnosed with Whipple's disease (WD) after several months of constitutional complaints and adenopathies that were initially misinterpreted as sarcoidosis. Initial treatment included doxycycline, hydroxychloroquine and prednisolone, which was suspended due to long-term clinical stability. Four months after prednisolone suspension, the patient presented with right periorbital oedema and erythema. Ophthalmological examination revealed restricted eye movements. A computed tomography (CT) scan demonstrated signs of myositis. The patient was treated with anti-inflammatory and antibiotic drugs, that induced remission of the orbitopathy. During the following two years, she presented three relapses, affecting both the right or the left eyes. The last episode was also associated with systemic corticosteroid tapering. Orbitopathy is a rare form of WD presentation and the diagnosis of this condition may be challenging. As the clinical spectrum may range from an incidentaloma to a severe compressive neuropathy, proper recognition and management of WD orbitopathy is essential.

Background: Little is known about long-term morbidity and mortality in Whipple’s disease (WD). Aim: To describe morbidity and mortality in patients with WD on a long-term follow-up. Materials & methods: Comorbidities, mortality and causes of death were retrospectively registered. Results: A total of 35 patients with WD (9F, 54 ± 11 years) were followed-up for a median of 104 months. Nine patients developed ten complications; three patients died. A total of 31 severe comorbidities apparently unrelated to WD were found in 20 patients: preneoplastic/neoplastic disorders in seven, thromboembolic and cardiovascular events in seven, pneumonia in four, candidiasis in ten patients. Conclusion: WD is frequently complicated by potentially life-threatening infectious, neoplastic and thromboembolic disorders, thus highlighting the need for a life-long multidisciplinary follow-up.