A 33-year-old male smoker with a history of allergy to acetylsalicylic acid and common variable immunodeficiency syndrome diagnosed at 6 years of age based on analytical criteria for childhood (IgA and IgM multiple-dose infection) and lower-than-
The patient had presented pulmonary bronchiectasis, nasosinusal polyposis, gastroesophageal reflux, platelet adhesion deficit, arthritis, cryptorchidism and phlebitis of the lower limbs and intervened hip.
She came to the emergency room for diarrhea, without blood or pathological products, accompanied by abdominal pain in the right iliac fossa of 5 months of evolution; she also reported loss of about 15 kg of weight in this period.
On physical examination, the patient was afflicted with auscultation, was hemodynamically stable. The patient presented with right iliac fossa pain without signs of peritonitis.
The hemogram showed hemoglobin of 11.5 g/dl, hematocrit 33 %, mean corpuscular volume of 73, leukocytes 25,800/mm3 (84 % segmented), platelets 516,000/mm3.
Thyroid biochemistry showed high levels of acute phase reactants (CRP 5.6 mg/dl, sedimentation rate 36 mm/h, alpha1glycoprotein acid 216 mg/dl and normal fecal calprotectin 1640 μg).
The proteinogram showed a moderate inflammatory profile with IgM 1 mg/dl (56-352 mg/dl), IgG 807 mg/dl (640-1.350 mg/dl), IgA 7 mg/dl (70-132 mg/dl).
The coprocultive, as well as serology for HBV, HCV and HIV, was negative.
ANA had negative antiDNA titers and negative antitransglutaminase IgG antibodies.
Mantoux was also negative.
Chest X-ray showed bronchiectasis in both lung fields as the only findings.
An intestinal transit and an abdominal CT were performed, which showed signs compatible with inflammatory bowel disease type Crohn's disease at ileal and cecal level.
The ileoscopy showed a cecal and ileal congestive mucosa with several superficial ulcers fibrinated on the surface, taking biopsies at both levels.
The anatomopathological study of the samples was informed of the mucosa of the large intestine with fibrinolytic material and granulation tissue, presenting architectural distortion of the crypts, without observing plasma cells.
Sputum mycobacterial culture and Zielh-Nielsen stain were negative in intestinal tissue samples.
All these data led to the diagnosis of Crohn-like disease in a patient with common variable immunodeficiency. Treatment with prednisone at a dose of 1 mg/kg of weight for 2 weeks was indicated, followed by progressive dose reduction.
But the patient had corticodependency, it was not possible to reduce the dose of 20 mg of prednisone a day, appearing clinical worsening manifested as recurrent abdominal pain and increased stools, so it was decided to start treatment with adalimumab 40 weeks.
The patient showed clinical improvement, yielding symptoms after several weeks of treatment, decrease of acute phase reactants (PCR 0.4 mg/dl and ESR 21 mm/h) and decrease of mural CT-screening level.
No adverse effects were observed during this period.
