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: Hemophagocytic Lymphohistiocytosis (HLH)

Figure 1. Axial (Panel A) and coronal (Panel B) PET-CT Scan Images showing numerous metabolically active pulmonary nodules and mediastinal lymph nodes.

Figure 2. Axial (Panel A) and Sagittal (Panel B) images of the chest CT showing consolidative pulmonary nodules with surrounding ground glass halo and mediastinal lymphadenopathy.

Figure 3. Bone Marrow Biopsy (x1000). Panel A: macrophage engulfing a neutrophil. Panel B: macrophage engulfing erythrocyte debris.

Figure 4. Panel A: Lower power view of the lung biopsy (H&E stain) showing the interface between the neoplastic lymphocytic infiltrate and benign, normal lung. Panel B: high power view showing the neoplastic B-cell lymphoma with sheets of large lymphocytes.

A 41-year-old African American woman with a history of diffuse large B cell lymphoma in remission was admitted to the hospital with severe dyspnea and abdominal pain. Recent imaging revealed extensive pulmonary and liver nodules with significant mediastinal lymphadenopathy (Figures 1 and 2).  She had an extensive outpatient evaluation of these abnormalities including multiple percutaneous and endoscopic biopsies which were nondiagnostic.  She deteriorated clinically and a ferritin level was elevated at 36,284 ng/mL.  Due to the markedly elevated ferritin, a bone marrow biopsy was performed and was normocellular with trilineage hematopoiesis and erythrophagocytosis consistent with hemophagocytic lymphohistiocytosis (HLH, Figure 3).  A VATS guided lung biopsy was performed revealing recurrence of the diffuse large B cell lymphoma (Figure 4).  She started chemotherapy with the E-SHAP (etoposide, methylprednisolone, cytarabine, cisplatin), however, became severely pancytopenic and developed acute respiratory failure, shock and multi-organ failure.  She died despite aggressive care in the intensive care unit.  Patients with HLH often present with sepsis like symptoms and multiorgan failure.  Measurement of serum ferritin level is a critical test in suggesting the diagnosis once infection is excluded.  Early recognition and prompt treatment is essential to preventing fatal outcomes.

Tauseef Afaq Siddiqi, MD¹; Carlos Tafich Rios, MD²; Carlos L Cantu, MD³; James Knepler, MD¹; Linda Snyder, MD¹

¹ Division of Pulmonary, Allergy, Critical Care and Sleep Medicine,

² Department of Medicine,

³ Department of Pathology, The University of Arizona, Tucson, AZ 85724, USA.

References

1. Raschke RA, Garcia-Orr R. Hemophagocytic lymphohistiocytosis: a potentially underrecognized association with systemic inflammatory response syndrome, severe sepsis, and septic shock in adults. Chest. 2011;140(4):933-8. [CrossRef] [PubMed] 
2. Okabe T, Shah G, Mendoza V, Hirani A, Baram M, Marik P. What intensivists need to know about hemophagocytic syndrome: an underrecognized cause of death in adult intensive care units. J Intensive Care Med. 2012;27(1):58-64. [CrossRef] [PubMed]

Reference as: 

Siddiqi TA, Rios CT, Cantu CL, Knepler J, Snyder L. Medical image of the week: hemophagoctyic lymphohistiocytosis (HLH). Southwest J Pulm Crit Care. 2013;7(6):351-2. doi: http://dx.doi.org/10.13175/swjpcc157-13 PDF