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Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) presents a significant medical challenge, with an annual incidence of 2.4 to 4.1 cases per 100,000 individuals and a prevalence of 29.6-54.6 cases per 100,000 individuals in the US. Diffuse alveolar hemorrhage (DAH) is a rare but grave complication of AAV, often associated with a mortality rate as high as 58.3%. This case study highlights a 52-year-old male patient who presented with hemoptysis and was diagnosed with DAH on bronchoscopy, subsequently testing positive for ANCA-PR3. Prompt intervention, including pulse steroids, rituximab, and plasmapheresis, along with the novel FDA-approved drug avacopan, led to significant improvement within four weeks. Early recognition and aggressive management are crucial in mitigating the life-threatening consequences of DAH in AAV, emphasizing the importance of bronchoscopy and advanced therapeutic modalities. This case underscores the potential efficacy of avacopan in managing ANCA vasculitis post-acute phase, offering hope for improved outcomes in this challenging condition.

Diffuse alveolar hemorrhage (DAH), a rare complication of coexisting antiphospholipid syndrome (APS) and systemic lupus erythematosus (SLE), poses significant diagnostic and therapeutic challenges, especially with recurrent episodes. We present a 27-year-old male with catastrophic APS and SLE who experienced acute respiratory failure and hemoptysis due to DAH. Despite aggressive therapy with immunosuppressants, plasma exchange, and anticoagulation, he had recurrent DAH episodes requiring repeated admissions. Early recognition, multidisciplinary management, and utilization of effective targeted therapies, such as intravenous immunoglobulin, in refractory cases are crucial for improving outcomes in this challenging complication.

BACKGROUND: Cryoglobulinemia with pulmonary involvement is rare, and its characteristics, radiological findings, and outcomes are still poorly understood.
METHODS: Ten patients with pulmonary involvement of 491 cryoglobulinemia patients at Peking Union Medical College Hospital were enrolled in this retrospective study. We analyzed the characteristics, radiological features and management of pulmonary involvement patients, and compared with those of non-pulmonary involvement with cryoglobulinemia.
RESULTS: The 10 patients with pulmonary involvement (2 males; median age, 53 years) included three patients with type I cryoglobulinemia and seven patients with mixed cryoglobulinemia. All of 10 patients were IgM isotype cryoglobulinemia. All type I patients were secondary to B-cell non-Hodgkin lymphoma. Four mixed patients were essential, and the remaining patients were secondary to infections (n = 2) and systemic lupus erythematosus (n = 1), respectively. Six patients had additional affected organs, including skin (60%), kidney (50%), peripheral nerves (30%), joints (20%), and heart (20%). The pulmonary symptoms included dyspnea (50%), dry cough (30%), chest tightness (30%), and hemoptysis (10%). Chest computed tomography (CT) showed diffuse ground-glass opacity (80%), nodules (40%), pleural effusions (30%), and reticulation (20%). Two patients experienced life-threatening diffuse alveolar hemorrhage. Five patients received corticosteroid-based regimens, and four received rituximab-based regimens. All patients on rituximab-based regimens achieved clinical remission. The estimated two-year overall survival (OS) was 40%. Patients with pulmonary involvement had significantly worse OS and progression-free survival than non-pulmonary involvement patients of cryoglobulinemia (P < 0.0001).
CONCLUSIONS: A diagnosis of pulmonary involvement should be highly suspected for patients with cryoglobulinemia and chest CT-indicated infiltrates without other explanations. Patients with pulmonary involvement had a poor prognosis. Rituximab-based treatment may improve the outcome.

A teenage girl with systemic lupus erythematosus (SLE) was admitted with fever, dry cough, and dyspnea on exertion. Chest computed tomography revealed bilateral diffuse infiltration and swelling of the mediastinal lymph nodes. The bronchoalveolar lavage (BAL) fluid was light red, suggesting diffuse alveolar hemorrhage (DAH). Therefore, glucocorticoid pulse therapy was initiated. However, blood and BAL fluid cultures showed the growth of Cryptococcus neoformans. The patient was diagnosed with disseminated cryptococcosis. The patient was treated with liposomal amphotericin B and flucytosine; the prednisolone dose was rapidly tapered. Infections should be thoroughly ruled out in patients with SLE and DAH.

Primary cardiac tumors are a rarity, and sarcomas emerge as the prevailing form of primary malignant cardiac tumors across age groups, encompassing both children and adults. Within this category, angiosarcoma stands out, constituting around 31% of all primary malignant cardiac tumors. Primary cardiac angiosarcoma displays a notably aggressive nature, characterized by early systemic metastasis, and is accompanied by a generally unfavorable prognosis. We describe a case concerning a previously healthy teenage girl who displayed persistent constitutional symptoms and hemoptysis for 15 days. Subsequent investigation uncovered alveolar hemorrhage, ultimately linked to a cardiac angiosarcoma. The difficulty in this instance arose from the vague nature of the initial symptoms, posing a challenge to promptly and accurately diagnose the condition. This case highlights the aggressive nature of primary cardiac angiosarcoma. The vague initial symptoms underscore the need for early detection and optimized treatment to improve the generally unfavorable prognosis associated with this condition. Increased awareness and a multidisciplinary approach are crucial in addressing the diagnostic and therapeutic challenges posed by primary cardiac angiosarcoma.

The association between antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis and hematologic malignancy has been previously described and remains a rare phenomenon (although potentially underdiagnosed). We report the case of an 81-year-old patient with myelodysplastic syndrome who was managed for an infectious-appearing pneumonia, which subsequently complicated into complete heart block and severe acute respiratory distress syndrome with a fatal outcome. The final diagnosis is severe hemorrhagic alveolitis due to ANCA-associated vasculitis meeting the criteria for microscopic polyangiitis. This article provides an opportunity to discuss the association between ANCA-associated vasculitis and hematologic malignancies and the adverse prognosis associated with it.

Anti-glomerular basement membrane (GBM) disease or Goodpasture syndrome is a rare disorder characterized by anti-GBM autoantibodies targeting the type 4 collagen of the basement membrane, resulting in rapidly progressive glomerulonephritis with or without alveolar hemorrhage. Pulmonary manifestations are less common in the elderly. Isolated pulmonary manifestations are rare in all age groups, and even more so in the elderly. We present the case of a lady in her late 70s, who presented initially with massive hemoptysis in the absence of renal dysfunction, which was presumed to be secondary to underlying bronchiectasis and infection. However, she later developed rapidly progressive acute kidney injury despite improvement in pulmonary symptoms and was diagnosed with anti-GBM disease. The delay in diagnosis and subsequent treatment due to the atypical presentation resulted in irreversible renal injury and the need for lifelong dialysis. This case demonstrates the need to consider atypical presentations of rare disorders, to ensure early diagnosis and optimal prognosis, especially when the clinical history cannot be explained by findings on examination and investigation.

Granulomatosis with polyangiitis (GPA) has a multitude of presentations, including appearing as a refractory community-acquired pneumonia (CAP) or an isolated localized pulmonary disease. This case describes a patient suspected to have a CAP before further workup and lung biopsy revealed his diagnosis of GPA. This case report demonstrates GPA\'s diverse presentations, critical complications such as diffuse alveolar hemorrhage (DAH) and cardiac tamponade, and nuanced management options.

To describe the pulmonary involvement in patients with catastrophic antiphospholipid syndrome (CAPS), focusing on its relationship with extrapulmonary involvement, laboratory, radiological, and pathological findings.
This retrospective cross-sectional study includes all patients grouped in the \"CAPS Registry\". All cases were reviewed, and those with pulmonary thromboembolism (PE) and/or diffuse alveolar hemorrhage (DAH) were selected. Data on pulmonary and extrapulmonary clinical presentation, radiologic patterns, laboratory findings, associated autoimmune diseases, treatments, and outcomes were analyzed. Frequency distribution and measures of central tendency were used to describe the cohort. Comparison between groups regarding qualitative variables was undertaken by chi-square or Fisher exact test, while T-test for independent variables was used to compare groups regarding continuous variables. IBM-SPSS v.22 was used for data analysis.
PE was reported in 129 (48.6 %) episodes, DAH in 75 (28.3 %) episodes, and overlap (DAH plus PE) in 7 (2.6 %) episodes. Bronchoalveolar lavage (BAL) was performed in 35 (4.9 %) CAPS episodes, and lung pathology samples were obtained in 84 (10.5 %) episodes (including autopsies). A significant relationship was observed between DAH and laboratory features of thrombotic microangiopathy (TMA). A meaningful relationship was also found between triple antiphospholipid antibody positivity and pathological TMA (26.5 %) as well as hypocomplementemia and DAH (24 %).
Pulmonary involvement may include both TMA and non-thrombotic inflammation, which can be differentiated into three patterns: PE, DAH with systemic TMA with hypocomplementemia or DAH without systemic TMA with/without hypocomplementemia.