BACKGROUND: Inflammatory Bowel Diseases (IBD) are rising in incidence in the pediatric population. While most present classically, some have limited findings. We present a case of pediatric Crohn's Disease (CD) diagnosed during a fever of unknown origin (FUO) evaluation. We aim to stress the importance of uncommon presentations of a diagnosis with significant implications and requiring prompt intervention. CASE: A 6-year-old male presented with 2 weeks of evening 104–106 F fevers, weakness, decreased intake, and weight loss. He also had one loose, non-mucous, non-bloody stool daily. Fevers failed to resolve with outpatient courses of antibiotics and CRP continued to rise. History revealed camping in North Carolina 2 weeks preceding symptom onset. Family history was unremarkable. No further symptoms, including abdominal pain, were elicited. Exam exhibited fever, tachycardia, tachypnea, uvular and palatal ulcers, dry mucous membranes, cervical, submandibular and inguinal lymphadenopathy. No skin, eye, joint, perianal or abdominal findings observed. Preliminary labs showed hypoalbuminemia (2.6), elevated CRP (7.4) and ESR (55), mild leukocytosis (14.9), and microcytic anemia (Hgb 10.1, MCV 71.8). An extensive FUO evaluation failed to identify a source and thus imaging was pursued. CT abdomen/pelvis showed terminal ileitis and free fluid along the right paracolic gutter and right hepatic margin. Endoscopic evaluation for IBD discovered esophagitis along with ulcerations, edema and friable mucosa in the terminal ileum. Pathology was significant for active chronic lymphoplasmacytic gastritis with eosinophils and subtle early granulomatous inflammation with multinucleated giant cells. Distal esophagus showed chronically active inflammation and lymphocytic esophagitis without granulomas. Terminal ileum biopsy revealed active, chronic granulomatous ileitis with ulceration and granulation. No organisms noted throughout. He was diagnosed with CD and initiated on steroids along with Mesalamine. DISCUSSION: One quarter of IBD is diagnosed by the age of 20, with 4% presenting before 5 and 18% before 10 years of age. Recent incidence reports show combined U.S. and Canadian data of 10 per 100,000. Prevalence in the U.S. alone is 100–200 per 100,000. Furthermore, IBD in those less than ten is becoming more common. With recent therapeutic advances, significant morbidity and mortality is preventable. However, treatment delays occur from a diagnostic lag in 20% when symptoms are ambiguous. This case highlights early onset IBD and brings attention to non-classic presentations. Our patient was diagnosed 1.5 months after initial symptoms. An average diagnosis lag time of about 3.4 months in pediatric CD has been documented, with further delay if pre-existing gastrointestinal symptoms exist (26 months). Whereas CD is considered when abdominal pain, diarrhea, and weight loss are identified, our patient presented with daily fever along with oral lesions, lymphadenopathy and weight loss. A few case reports cite FUO, another difficult and often unclear diagnosis, as the presentation for IBD. Fever as the sole initial symptom for IBD occurs in 10-15% of cases. IBD is a complicated illness, especially in the pediatric population. When considering effects on growth, psychosocial development and the long-term complications, we must consider IBD in the differential of pediatric patients with vague and persistent symptoms including FUO.
CASE: Inflammatory Bowel Disease (IBD) is becoming more common in an increasingly diverse population. Exposure history is important, especially when prescribing immunosuppressive therapy. We present a case of suspected disseminated histoplasmosis in a gentleman with longstanding Ulcerative Colitis (UC) on anti-TNF with an atypical, large, non-healing duodenal ulcer. We aim to highlight risks, presentation, and management of histoplasmosis in IBD patients on immunosuppression with anti-TNFs. A 49-year-old-male with a 21-year history of left sided UC in remission on Infliximab (10 years) presented to our ED with orthostatic symptoms and melena. He reported two months of heartburn and epigastric pain refractory to acid suppression. In the ED, vitals were unremarkable. Labs showed BUN 38 mg/dL, hemoglobin 13.3 g/dL, and abnormal AST/ALT. Evaluation of mild chest discomfort revealed normal EKG and calcified nodule in the left lower lobe on chest X-ray. Tuberculosis testing was negative. EGD found a massive, 3-4cm, cratered, medial wall, hemi-circumferential ulcer from duodenal apex into the second duodenal segment (D2). Biopsies revealed acute inflammation, without CMV, dysplasia, malignancy or helicobacter pylori. CT identified a large mass 5x3.1x3.2cm in the pancreaticoduodenal groove from D2 without pancreatic duct dilation. There were prominent right axillary and sub-pectoral lymph nodes and the calcified granuloma seen on X-ray. He denied NSAID use. Symptomatic improvement occurred on aggressive acid suppression. EGD a month later showed persistent ulcer with unchanged pathology. EUS showed significant peri-duodenal thickening without malignant findings. IgG/IgG4 immunostains were negative. CEA and CA 19-9 were normal. Subsequent EGDs and imaging showed no changes. He developed duodenal stenosis requiring dilation. Hematology/Oncology evaluation was unrevealing and hyper-secretory disorder was ruled out. Lack of healing over seven months prompted referral to Infectious Disease. They identified bird dropping exposure with repeated deck pressure washing. Positive Histoplasma immunodiffusion M band indicated prior infection. Given exposure, lab, chest imaging and endoscopic findings, treatment for disseminated histoplasmosis (DH) with Itraconazole was initiated. Infliximab was held and mesalamines were restarted. Histoplasmosis is endemic to the Ohio/Mississippi River Valley and other countries. Disseminated histoplasmosis, typically found in the immunocompromised, presents in many ways with GI involvement in 70%. Diagnosis can be difficult as histoplasmosis mimics other diseases, including IBD. Prognosis is poor if left untreated. Endoscopically, ulcerations, mass-like lesions, or strictures are seen. Aside from identifying yeast, pathology is nonspecific. Severity guides treatment, classically involving Itraconazole. In IBD and diseases managed with immunosuppression (e.g. anti-TNFs), stopping therapy during infections is standard of care. Therapy may resume after treatment response. Treatment may be a year for DH. Prophylaxis for histoplasmosis, the most common fungal infection with anti-TNF use, is controversial. Literature exists where anti-TNF was continued during treatment of histoplasmosis with good outcomes. There were no recurrences with continuation or re-initiation of anti-TNF after treatment. However, many patients switched therapies. Though histoplasmosis rarely causes infection in IBD patients, outcomes can be poor. We must be aware of possible exposures, atypical or presentations mimicking IBD to identify infection early, stop immunosuppression and provide timely treatment.
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