JOURNAL OF THE LOUISIANA STATE MEDICAL SOCIETY
was not previously compliant. Rheumatology was consulted, but, although the diagnosis of BD was possible, at this time the patient did not fulfill definite diagnostic criteria.
once for tracheoplasty for treatment of tracheomalacia, and the second time for treatment of community-acquired pneumonia that failed oral antibiotics.
COMMENTARY
CASE 2
Behçet's Disease was first described by a Turkish dermatologist, Dr. Hulusi Behçet, in 1937 (the original article has been reviewed in reference one and translated in reference two), as a syndrome of recurrent oral and genital ulcers associated with uveitis. Since then, BD has been recognized as a multi-systemic vasculitis. The new International Criteria for Behçet’s Disease (ICBD) do not require recurrent oral and genital aphthosis for the diagnosis.3 The cases reported here illustrate some of the common challenges clinicians face when managing patients with inflammatory diseases. In the first case, the clinicians were faced with a common dilemma when caring for patients with rheumatic conditions: to decide which manifestations of inflammation are due to the underlying autoimmune process, and which may be caused by opportunistic infections in the settings of immune dysfunction and immunosuppressive therapies. While her treating team was facing the delicate balance of adjusting immunosuppressive therapy during reactivation of pulmonary tuberculosis, they were surprised with an atypical presentation of autoimmune disease in an organ close to the source of infection, with resulting overlapping of clinical syndromes and a delayed diagnosis and treatment. Laryngeal involvement in BD has been seen sparingly in medical literature, but there is evidence that it may occur in a significant proportion of cases. Webb et al.4 reviewed the medical literature on otolaryngological manifestations of BD. They report cases of pharyngolaryngeal stenosis, but also review reports on hearing loss, vestibular involvement, and sinusopathy. In a northern European cohort, 5 out of 15 patients had laryngeal involvement.1 Our patient required a temporary tracheostomy, which was further complicated by tracheomalacia. One case report demonstrated the possibility of avoidance of surgery in BD-associated laryngeal stenosis with aggressive immunosuppression with pulse methylprednisolone and infliximab,5 underscoring the importance of early and accurate diagnosis of laryngeal BD. Other important possibility to be considered would be that the cavitary lung lesion was a parenchymal involvement by BD itself, and not Mycobacterium tuberculosis infection. Parenchymal BD may indeed present as cavitary lesions.6 Although the infection was not microbiologically proven, tuberculosis was still considered more likely because the patient was treated in a country with high prevalence of tuberculosis (Brazil), the presentation and imaging were considered typical, and the pulmonary lesions improved with anti-tuberculosis regimen, before the intensification of steroid therapy.
A 55-year-old man from Jordan presented with hemoptysis and acute respiratory failure. He was intubated and subjected to diagnostic bronchoscopy, which showed mucosal petechiae but no other lesions. His past medical history is significant for panhypopituitarism and central sleep apnea after a remote head trauma, obesity, type 2 diabetes mellitus with severe peripheral neuropathy treated with an implanted hydromorphone pump, and new diagnosis of hepatic cirrhosis secondary to non- alcoholic steato-hepatitis. After being extubated, patient could give a detailed history that he had been losing weight for three months and had a persistent penile ulcer. Social history was significant for 15 pack-year smoking history and no history of alcohol or drug use. On physical exam, the patient had one aphthous oral ulcer and one penile ulcer on the glands. He also had multiple scattered lesions over both arms with signs of inflammation and impaired healing, corresponding to sites of previous intravenous lines and blood draws. These skin lesions may have been a pathergy phenomenon. HLA-B51 was not obtained during the hospitalization. Bronchoalveolar lavage was negative for infectious etiologies. Platelet count was mildly decreased at 135,000/mcL. Both the international normalized ratio and the activated partial thromboplastin time were within normal limits. Von Willebrand factor analysis was normal. Platelet function assay was performed and was not suggestive of platelet dysfunction. Thromboelastography showed normal results. Anti-nuclear antibody, rheumatoid factor, anti-neutrophil cytoplasmic antibody, anti-glomerular basement membrane were all negative. Serologies for infectious etiologies were similarly negative, including human immunodeficiency virus, rapid plasma reagin, cytomegalovirus, Epstein-Barr virus, Varicella- Zoster virus. Quantiferon Gold was negative. Penile swab for Chlamydia and Gonorrhea DNA was negative. Computed tomography scan of the chest with intravenous contrast was significant for bilateral small pulmonary nodules and ground glass opacities. Ultrasonography revealed a coarse liver with parenchymal disease. Further work up revealed Hepatitis B and C serologies negative, normal ferritin, normal ceruloplasmin, normal alpha-1-antitrypsin. Also, patient showed no clinical signs of decompensation from cirrhosis, including no ascites, no encephalopathy, and no coagulopathy except for mildly decreased platelet count as above. Patient progressed with gradual improvement until he had no further hemoptysis. He had been started on oral hydrocortisone for treatment of panhypopituitarism, a treatment to which he
J La State Med Soc VOL 170 MAY/JUNE 2018 77
Made with FlippingBook Digital Publishing Software