A 39-year-old man diagnosed in childhood with cystic fibrosis (genotype 3849 + 10 kb leading to a C to-T change, homozygous) had been followed at a CF specialty clinic but had significant gaps between visits. The patient presented with a three-day history of dyspnea, productive cough, fevers, and hypoxia. Prior to hospitalization patient was on as needed oxygen at night, but had increased to 2.5 lpm at home over the past three days. Chest radiograph showed new bilateral perihilar infiltrates and laboratory evaluation showed leukocytosis (14 x 103 cells/muL). The patient had a history of colonization with methicillin-resistant Staphylococcus aureus and Pseudomonas aeruginosa, chronic sinusitis, obesity, and gastric reflux disease. Patient did not have history of pancreatic insufficiency prior to hospitalization. Three months prior to hospitalization, pulmonary function tests were FVC 3.60L/76% predicted, FEV1 2.18L/57% predicted, FEV1/FVC 60/75% predicted, FEV 25-75 30% predicted; oxygen saturation was 93% on room air. At that time patient was also diagnosed with thrush and tinea cruris. Prior to that visit there was a 3-year gap in care. At the time of hospitalization home medications included albuterol/ipratropium inhalation solution, azithromycin, docusate, dornase alfa, ergocalciferol, fluticasone/salmeterol inhalation powder, multivitamin, pantoprazole and tobramycin inhalation solution. The patient denied tobacco, alcohol, or recreational/injection drug use. He was in a monogamous relationship with a female partner. The patient denied history of having sexual contact with men. The patient also had no history of blood transfusions. Patient did report that a former female partner died of an unknown illness several years prior to his hospitalization.
The patient was admitted to the hospital with a presumed diagnosis of CF exacerbation. Initially the patient received 5 lpm of oxygen and oxygen saturation was 94%. Intravenous cefepime, tobramycin, vancomycin and standard CF therapies were initiated. Infectious Diseases and Pulmonary services were consulted. Evaluations for causes of acute hypoxic respiratory failure included bacterial, fungal, and mycobacterial sputum cultures, mycoplasma and coccidioides serologies, screening for connective tissue disease, urine legionella antigen, urine pneumococcal antigen, and respiratory viral panel, all of which were unrevealing. An echocardiogram showed normal left ventricular systolic function (LEVF) with pulmonary artery pressure of 37 mmHg. The patient's pulmonary condition worsened; on hospital day 4 he developed respiratory distress and sudden increase in oxygen requirement. A computed tomography (CT) scan of the chest with contrast showed extensive bilateral parenchymal ground glass opacities with peribronchial consolidation, read as more consistent with atypical pneumonia than with CF exacerbation, though pre-existing bronchiectasis made interpretation difficult. The patient was transferred to the intensive care unit (ICU) (see Fig. 1). On hospital day 5 a consultant suggested HIV testing, as well as a bronchoscopy, but the patient was initially unstable and the procedure was deferred. On hospital day 8, an HIV screen was positive and confirmed with Western blot; CD4+ cell count was 56 cells/muL and HIV viral load was 947,000 copies/mL. On hospital day 9, the patient underwent bronchoscopy; pneumocystis was identified by calcofluor stain from bronchoalveolar lavage. The patient was treated with sulfamethoxazole/trimethoprim 15mg/kg/day (based on trimethoprim component), prednisone taper, and antiretroviral therapy. The patient responded to treatment and did not require intubation; however, he developed a spontaneous pneumothorax, requiring multiple thoracostomy tubes (one with a Heimlich valve) and eventually was transferred to a specialty hospital for chemical pleurodesis. Screening tests for sexually transmitted diseases, tuberculosis, hepatitis, and toxoplasmosis were negative.
The patient is now followed by both the HIV and CF specialty clinics at our institution and he has remained stable with chronic oxygen requirement (2-3 LPM), improved CD4 cell count and an undetectable HIV viral load. No evidence of prior HIV screening was documented prior to hospitalization. His current female partner is not infected with HIV.