Case Presentation: Granulomatosis with Polyangiitis (GPA) is a rare necrotizing vasculitis characterized by chronic granulomatous inflammation and localized and systemic manifestations. It typically involves the upper airway, lungs, and kidneys, but potentially affects every organ with a highly variable clinical trajectory. Diffuse alveolar hemorrhage (DAH) is one of its least common pulmonary manifestations, characterized by hemoptysis, anemia, and diffuse alveolar infiltrates as well as hypoxemic respiratory failure. Owing to the variable presentation, the disease may elude accurate diagnosis, presenting diagnostic and therapeutic challenges.A 74-year-old Caucasian male with a history of atrial fibrillation and heart failure presented with progressively worsening generalized swelling and shortness of breath over months not improving with cardiology management and hemoptysis that required pulmonologist care. Imaging studies including chest radiography and CT scan with contrast revealed bilateral interstitial patchy opacities at lung bases predominant on the right lung with moderate right-sided pleural effusion and atelectasis. Pulmonary function tests revealed a restrictive ventilatory and diffusion defect. Labs were positive for mild anemia with a hemoglobin of 12.5 mg/dl and the presence of anti-neutrophil cytoplasm antibodies, while anti-proteinase-3 and anti-myeloperoxidase antibodies were negative. Bronchoscopy demonstrated blood throughout the bronchial tree, primarily on the right bronchial side, without identifiable bleeding site and hemorrhagic bronchoalveolar lavage (BAL) with mild chronic inflammation. Cytology ruled out malignant cells, fungal hyphae, and acid-fast bacillus. Subsequent wedge biopsy sent to Mayo Clinic revealed an acute and organizing DAH with organizing fibrinous pleuritis, that was highly suspicious for active capillaritis related to ANCA-associated vasculitis syndrome with final diagnosis of GPA with DAH, medically improving after chest tube insertion with drain placement that helped drain the effusion and treatment with rituximab and corticosteroids.
Discussion: GPA is a highly variable disease and is often misdiagnosed and mismanaged, especially in rare presentations like this case. The presentation of GPA with DAH is the least common only observed in about 5-15% of cases. The DAH ‘triad’ of hemoptysis, new infiltrates on imaging, and anemia is seen only in 34% of cases.
Conclusions: The presence of anemia is commonly associated with DAH while hemoptysis is only noted in one-third of the cases. DAH typically exhibits nonspecific clinical and imaging findings, and the most valuable diagnostic clue is the retrieval of bright red fluid during BAL. Thus, given its elevated risk of mortality, it’s important to maintain a low threshold for suspecting GPA, and physicians should also remain attentive to its unconventional presentations, such as DAH.