JOURNAL OF THE LOUISIANA STATE MEDICAL SOCIETY
Diagnostic Challenges in Rheumatic Diseases: Commentary on Two Case Reports
Ivan Saraiva, MD, Goutham Talari, MD
Two cases of Behcet's disease are presented. Case 1 shows a young woman with pulmonary embolism, skin ulcers that developed pulmonary tuberculosis as a complication of treatment, and later developed laryngeal involvement that was unclear if due to Behcet's or to opportunistic tuberculosis infection. Case 2 shows a Middle Eastern male with non-alcoholic steato-hepatitis who presented with hemoptysis and mucosal ulcers. Extensive work up was performed and Behcet's remained the most likely possibility to explain his findings. A commentary on these specific complications of Behcet's disease is provided.
CASE REPORTS
prednisone 0.5mg/kg daily and colchicine 0.5mg twice daily.
CASE 1
HLA-B51 came back negative. Nevertheless, patient improved quickly with healing of skin ulcers and disappearance of erythema nodosum. She reported increased general wellbeing and energy. Two weeks later, patient developed a dry cough. The cough worsened for a few days, and a chest radiography revealed a cavitary lesion of the right upper lobe, confirmed by computed tomography. There was no sputum production so acid-fast stain and mycobacterial culture could not be performed. PPD showed induration of 25mm. Prednisone was slowly tapered down and treatment was started for culture- negative pulmonary tuberculosis with rifampin, isoniazid, pyrazinamide and ethambutol (RIPE). While she was on in her first week of RIPE therapy, patient was admitted to hospital for worsening respiratory symptoms. A laryngoscopy was performed by an otolaryngologist, which showed inflammation “suggestive of laryngeal tuberculosis.” The patient was discharged home with treatment regimen unchanged, after being stable for a period of observation. The patient continued to complain of dry cough and shortness of breath. She presented to the emergency room about two weeks later in acute respiratory distress. She was diagnosed with upper airway obstruction and an urgent tracheostomy was performed. Repeat computed tomography of the chest showed partial improvement of lung lesions. Pathology report, from a biopsy which had been collected from the larynx, failed to reveal tuberculosis. The laryngitis was now thought to be secondary to BD. At this time prednisone dose was increased to 1mg/kg. Eventually, the patient improved clinically, completed a six- month tuberculosis treatment, was decannulated from the tracheostomy, and eventuallymade a full recovery. During follow up for the next four years, shewas admitted to the hospital twice,
A 32-year-oldwoman fromBrazil presented initiallywith bilateral pulmonary emboli with pulmonary infarcts. She endorsed a past medical history of pituitary adenoma, and a family history significant for deep venous thrombosis in her grandmother. She had used oral contraceptive pills, which were stopped after the emboli were diagnosed. There was no history of use of alcohol, tobacco, or drugs. She was started on anticoagulation with low molecular weight heparin, followed by oral warfarin. About one month after the diagnosis, the patient developed a necrotic skin lesion on her right foot. The ulcer was refractory to antibiotics. Laboratory tests were significant for normal blood cell count, normal metabolic panel, negative rheumatoid factor, negative anti-nuclear antibody, negative anti-neutrophil cytoplasmic antibody, normal complement levels, negative D-dimer, but a persistently elevated C-reactive protein at 20.4 to 29 mg/L for several months. A biopsy was obtained, and the pathology report described “chronic active cutaneous ulcer, nonspecific.” At this point, one possibility for diagnosis was an atypical presentation of protein C deficiency with the warfarin-induced skin necrosis, given the time with starting warfarin therapy. Patient was transitioned to anticoagulation with dabigatran (at that time this was the only non-vitamin K antagonist oral anticoagulant available, and it was used off label in this patient). The ulcer persisted, and new similar ulcerative lesions started to appear on both legs, as well as erythema nodosum. This time biopsy was resulted as “granulomatous vasculitis” after being reviewed by two pathologists. Patient eventually developed ulcers in both oral and genital mucosae. This was about seven months after the initial presentation. A working diagnosis of Behçet’s disease (BD) was proposed, and patient was started on
76 J La State Med Soc VOL 170 MAY/JUNE 2018
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