Case Presentation: A 42 year old male with no past medical history who worked as a firefighter and was a non-smoker presented with hemoptysis, chest pain, dyspnea requiring 2-3 liters of oxygen, dark urine, migratory joint pain, and constitutional symptoms for two weeks. The prior month, he fought wildfires in the California desert, where he first reported blood-tinged sputum. After poor response to antibiotics outpatient, he was treated inpatient at a different hospital for community acquired pneumonia with broad-spectrum antibiotics including meropenem and linezolid. Given lack of improvement and consideration of fungal pneumonia, bronchoscopy and bronchoalveolar lavage (BAL) were performed at the other hospital, with results pending. On transfer to our hospital, labs were significant for leukocytosis (18.46 K/uL), normocytic anemia (9.4 g/dL), and elevated alkaline phosphatase (182 U/L) and chest xray was significant for right mid lung infiltrates compatible with pneumonia. During admission, a broad differential was considered, and laboratory workup for fungal infection and autoimmune conditions was sent. On hospital day 2, the patient was treated with empiric fluconazole for Coccidioides pneumonia. CTA chest obtained from the prior hospital showed a masslike consolidation in the right lower lobe with satellite foci in the right upper lobe. Repeat CT chest abdomen pelvis from the prior hospital three days later showed patchy and consolidative bilateral airspace opacities, more extensive than prior. On hospital day 3, the outside BAL resulted, showing no fungal organisms, and biopsy revealed abundant acute and chronic inflammation. On the same day, a positive proteinase-3 (PR3) resulted. On hospital day four, a positive cytoplasmic antineutrophil cytoplasmic antibody (c-ANCA) resulted, and overnight, he desaturated to 85%, with chest xray showing increased bilateral mid lung hazy opacities. A repeat BAL for worsening infiltrates and anemia revealed diffuse alveolar hemorrhage (DAH). He was promptly transferred to the intensive care unit (ICU), where he was intubated for seven days and received pulse dose steroids and cyclophosphamide/mesna induction. A clinical diagnosis of granulomatosis with polyangiitis (GPA) was made. His ICU course was complicated by acute kidney injury, bilateral soleal deep vein thromboses, and fevers with bronchial culture positive for Pseudomonas requiring piperacillin-tazobactam. After extubation, he was initiated on avacopan and a steroid taper, with symptomatic improvement over five days.
Discussion: DAH is a rare presenting symptom of GPA, often requiring ICU-level care. In this case, given a characteristic history of travel to the California desert and lack of response to broad-spectrum antibiotics, the differential diagnosis was broad and included Coccidioides fungal pneumonia as well as potential autoimmune disease. Therefore, a broad workup was done at time of admission to investigate potential causes of hemoptysis with constitutional symptoms in a previously healthy patient, which facilitated a diagnosis of GPA.
Conclusions: A thorough workup is necessary at time of presentation for infectious and autoimmune causes of hemoptysis, lung imaging findings concerning for pneumonia with poor response to antibiotics, and escalating oxygen regiments. As labs may take time to result, timely laboratory tests and a pulmonary consult should be initiated to guide further management.