A 47-year-old man with a 17-year history of Behcet's disease with mucocutaneous and intestinal involvement presenting incomplete response to colchicine, corticosteroids, azathioprine, and sulfasalazine.
Initially medicated with colchicine and corticosteroids without complete resolution of complaints and additionally medicated with thalidomide with a total resolution of the symptomatology that, unfortunately, had to be suspended due to the side effect of sensitive polyneuropathy of the lower limbs. Corticosteroid dosage was escalated and azathioprine was introduced but without complete control of the complaints - the patient maintained oral and genital ulcers as well as asthenia. Sulfasalazine was also introduced because of intestinal manifestations such as abdominal pain, diarrhea, and hematochezia but without satisfactory resolution of the complaints.
Faced with this resistance, the patient was treated with adalimumab resulting in a rapid and complete resolution of the symptoms. Six months after initiation of treatment and about three days after the administration of another dose of adalimumab, the patient started to complain of fever with night and evening predominance associated with flank, chest, and retrosternal myalgias and inflammatory arthralgia of the shoulders, elbows, knees, and metacarpophalangea joints. He also reported dizziness, headache, and mechanical neck pain. He had nausea without vomiting.
Physical examination revealed oropharynx without thrush or ulcers. No cervical or axillary adenopathies on palpation. No signs of arthritis. Lower limbs with slightly swollen knees. No alterations in ophthalmologic or neurological exams and in cardiopulmonary evaluation.
Blood test results: Creatinine 0.74 mg/dL, Sodium 142 mmol/L, Potassium 4.0 mmol/L, CK 29 U/L, PCR 8.74 mg/dL Leukocytes 7,200; Neutrophils 61.1%; Lymphocytes 22.0%; Hemoglobin 14.2 g/dL, VGM 91.3 fL, Platelets 270,000; SR 32 mm/h.
Chest X-ray: perihilar reinforcement.
Serologies were performed for Cytomegalovirus, Epstein-Barr Virus, Herpes simplex 1 and 2, Chlamydia trachomatis, Coxiella burnetii, Mycoplasma pneumoniae, Clamydophila pneumoniae which were negative. Mantoux test and IGRA were also negative. Bronchoalveolar lavage, bacteriological examination, and mycobacterial tuberculosis detection by polymerase chain reaction and cultures were negative. Negative hemocultures.
Therapy with adalimumab was suspended and empirical medicated with ceftriaxone and azithromycin started with initial improvement of complaints.
About 2-3 days after the end of the antibiotic therapy, the patient started complaining of a continuous right chest pain, grade 6/10, which aggravated with mobilization and with right lateral decubitus position. He reported dry cough and night sweats, without fever. Appearance of erythema nodosum in both lower limbs.
Chest radiography revealed bronchopneumonia of the right upper lobe. Analytically with PCR 38.5 mg/dL, SR 47mm/hr, and increased anti-Legionella pneumophila IgM antibody titer from 1/320 to 1/1000.
Because no imaging improvement occurred, a high-resolution chest CT was performed that demonstrated nodulation reaching the right upper lobe, middle lobe, and lower lobes. Central permeable tracheobronchial tree. Multiple mediastinal and hilar adenomegalies reaching about 15 mm short axis. There was a small volume of bilateral pleural effusion.
Legionella pneumonia was assumed and was treated with clarithromycin for 10 days. There was improvement in complaints, only right chest pain maintaining.
Thoracic CT was repeated one month later, which revealed a reduction in adenomegaly. Micronodulation was dispersed in both lungs. Analytically, it had ECA 132 U/L (8-52 U/L). A mediastinal ganglion biopsy was performed that revealed confluent, non-necrotizing epithelioid granulomas and multinucleated Langhans and Touton giant cells, suggesting the diagnosis of sarcoidosis.
He was medicated with methylprednisolone 40mg for 6 months. CT imaging showed complete resolution of nodular lesions and adenopathies.