Medical Image of the Week: Diffuse Alveolar Hemorrhage in a Patient with ANCA Vasculitis and IgG4-Related Disease

Figure 1. Well-circumscribed, high-density, airspace opacities with a “crazy-paving” pattern in the upper and lower lobes with peripheral sparing (blue arrows) consistent with alveolar hemorrhage.

Figure 2. Well-circumscribed, high-density, airspace opacities with a “crazy-paving” pattern in the upper and lower lobes with peripheral sparing (blue arrows) consistent with alveolar hemorrhage.

The patient is a 47-year-old man with a history of bilateral orbital pseudotumor associated with immunoglobulin G4-related disease (IgG4-RD). He presented with progressively worsening exertional dyspnea evolving into multisystemic failure.  During the hospitalization, the patient was found to have pauci-immune ANCA-positive vasculitis and glomerulonephritis.

CT images (Figures 1 and 2) show relatively well-circumscribed and extensive upper lung predominant airspace opacities with high attenuation, in some cases with a patchy configuration. A background of interstitial prominence was also noted resulting in a "crazy paving" pattern”, consistent with diffuse alveolar hemorrhage.  This was confirmed with bronchoalveolar lavage.

Discussion

IgG4-RD (IgG4 related disease), is an autoimmune condition capable of causing inflammation and fibrosis of multiple organs, most classically the pancreas (1). IgG4 is the least abundant IgG in the serum and the least likely to stimulate immune activation due to its inability to activate complement (2).

The thoracic manifestations that have been described in cases of pure IgG4-RD include solid nodules, which can appear similar to malignant lesions. Interstitial changes have also been described in the form of non-specific interstitial pneumonia pattern, organizing pneumonia, bronchiolitis obliterans, acute interstitial pneumonitis and a sarcoid-like reaction. There may also be pleural involvement and thickening/irregularity of the central airways. The multiple varying presentations and their potential concomitance can lead to misinterpretation of findings (1-2).

This patient presented with the known history of IgG4-RD. The acute symptoms included hemoptysis/diffuse alveolar hemorrhage and renal failure. To the best of our knowledge, pulmonary hemorrhage has not been described as a potential manifestation of this IgG4-RD.   Therefore, the later diagnosed concomitant ANCA paucimmune vasculitis, likely explained the observed pulmonary findings. The coexistence of two different autoimmune vasculitides has been described before, both contributing to multiorgan-involvement (3).

Mariam Mostamandy BS and Diana Palacio MD

Department of Medical Imaging

The University of Arizona – Banner Medical Center

Tucson, AZ

References

1. Kurowecki D, Patlas MN, Haider EA, Alabousi A. Cross-sectional pictorial review of IgG4-related disease. Br J Radiol. July 2019:20190448. [CrossRef] [PubMed]
2. Campbell SN, Rubio E, Loschner AL. Clinical review of pulmonary manifestations of IgG4-related disease. Ann Am Thorac Soc. 2014;11(9):1466-75. [CrossRef] [PubMed]
3. Carruthers I, Shingare S, Khosroshahi A, et al. IgG4 plasma cell infiltration in granulomatosis with polyangiitis (formerly Wegener’s) lung biopsies. 2012 ACR/ARHP Annual Meeting. [Abstract 1534].

Cite as: Mostamandy M, Palacio D. Medical image of the week: diffuse alveolar hemorrhage in a patient with ANCA vasculitis and IgG4-related disease. Southwest J Pulm Crit Care. 2020;20(3):98-9. doi: https://doi.org/10.13175/swjpcc009-20 PDF 

Medical Image of the Week: Fat Embolism Syndrome

A 33-year-old man presented to the emergency department with shortness of breath and hemoptysis. He was discharged two days prior after hospitalization for a motor vehicle accident, in which he suffered a fracture of the shaft of the right femur. He had undergone open reduction and internal fixation of the fracture four days prior to this admission. He had diffuse parenchymal disease on his admission chest x-ray. A CT scan of the chest demonstrated multilobar ground glass opacities (Figure 1).

Figure 1. Thoracic CT scan showing ground glass opacities.

Bronchoscopy demonstrated progressively bloody BAL aliquots in two different lobes, consistent with diffuse alveolar hemorrhage (DAH). His workup for other etiologies was negative, and he was given a diagnosis of DAH secondary to fat embolism syndrome.

Joshua Malo, MD and Kenneth S. Knox, MD

Division of Pulmonary, Allergy, Critical Care, and Sleep Medicine

University of Arizona, Tucson, AZ

Reference as: Malo J, Knox KS. Medical image of the week: fat embolism syndrome. Southwest J Pulm Crit Care. 2014;8(4):246. doi: http://dx.doi.org/10.13175/swjpcc041-14 PDF

Medical Image of the Week: DAH

 

Figure 1. Portable CXR (A) and CT (B) showing diffuse infiltrates of unclear etiology.

Figure 2. Progressively bloodier lavage indicative of pulmonary hemorrhage syndrome.

A 59 year old female was admitted to the ICU with hypoxemic respiratory failure and a clinical picture of ARDS (Figure 1), requiring intubation and mechanical ventilation. She underwent bone marrow and renal transplantation several years prior for multiple myeloma and myeloma kidney, respectively. She had been restarted on lenalidomide one month prior to presentation.  She was also taking tacrolimus, mycophenolate, prophylactic antimicrobials, warfarin for deep venous thrombosis, and aspirin for coronary artery disease. Emergent bronchoscopy with bronchoalveolar lavage revealed progressively bloodier specimens (Figure 2) consistent with diffuse alveolar hemorrhage (DAH). Further work-up was negative for vasculitis. Her lenalidomide, anticoagulation, and trimethoprim/sulfamethoxazole was stopped. She was started on high dose steroids and improved over 2 weeks.

Sage P. Whitmore, MD; Candy Wong, MD; James L. Knepler, MD and Carmen Luraschi-Monjagatta, MD

Division of Pulmonary and Critical Care Medicine

Arizona Respiratory Center

University of Arizona

Tucson, Arizona

Reference as: Whitmore SP, Wong C, Knepler JL, Luraschi-Monjagatta C. Medical image of the week: DAH. Southwest J Pulm Crit Care 2013;6(3):129. PDF