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 

February 2018 Imaging Case of the Month

Michael B. Gotway, MD

Department of Radiology

Mayo Clinic Arizona

Scottsdale, AZ USA

Clinical History: An 18-year-old woman with a questionable history of asthma (one physician source claimed no clear history of asthma, whereas another source claimed severe asthma) presented to the emergency room with worsening shortness of breath and cough. The patient’s past medical history was otherwise largely unremarkable. She did have complaints of recurrent rhinorrhea and allergies, for which sinus CT (Figure 1) had been performed.

Figure 1. Unenhanced axial sinus CT shows multifocal sinus opacification (arrow = maxillary sinuses, arrowheads = ethmoid sinuses, double arrowhead= sphenoid sinus)

Physical examination was remarkable for coarse, right-greater-than-left basal rales and coarse breath sounds. The patient’s oxygen saturation was 98% on room air. Her nasal septum appeared deviated. The patient’s vital signs were within normal limits and she was afebrile.

Laboratory evaluation showed a normal complete blood count, electrolyte panel, and liver function tests. A digital frontal chest image (Figure 2) obtained at presentation is shown, with a comparison chest radiograph from 5 months earlier also shown.

Figure 2. A: Digital frontal chest image. B: Chest radiograph from 5 months earlier.

Which of the following represents the most accurate assessment of the frontal chest imaging findings? (Click on the correct answer to proceed to the second of seven pages)

1. Chest frontal imaging shows basilar fibrosis
2. Chest frontal imaging shows mediastinal and peribronchial lymphadenopathy
3. Chest frontal imaging shows multiple, bilateral small nodules
4. Chest frontal imaging shows normal findings
5. Chest frontal imaging shows patchy nodular opacities in the right lung

Cite as: Gotway MB. February 2018 imaging case of the month. Southwest J Pulm Crit Care. 2018;16(2):67-75. doi: https://doi.org/10.13175/swjpcc019-18 PDF

Medical Image of the Week: Levamisole-Induced Vasculitis

Figure 1. Purpuric lesion on the auricle of the ear.

Figure 2. Diffuse purpuric lesions in retiform patterns of the trunk and extremities.

A 51-year-old Hispanic woman presented with a worsening, diffuse, painful purpuric rash over the last 3 months, involving both auricles of the ears, both lower extremities, and her trunk. She also reported purulent discharge drainage from one of the lesions on her right posterior thigh, associated with fever and chills for one day. She was a daily cocaine user for the last 30 years.

On examination, she was febrile and tachycardic. There were diffuse retiform like non-blanching purpuric lesions without necrosis on ears, her lower extremities, and trunk (Figure 1 and 2). There was an open wound on the posterior aspect of her right thigh that had purulent drainage.

Laboratory investigations revealed neutropenia, normal complete metabolic panel, high erythrocyte sedimentation rate (ESR) and high C-reactive protein. Further autoimmune work-up revealed positive perinuclear antineutrophil cytoplasmic antibodies (p-ANCA), high anti-myeloperoxidase antibodies (MPO) titer, elevated IgM anticardiolipin antibodies, negative antinuclear antibodies (ANA), and low complement levels. HIV, hepatitis viral panel and cryoglobulin were negative. Urine toxicology was positive for cocaine. Benzoylecgonine, m-OH-benzoylecgonine, and cocaethylene, which are cocaine metabolites were detected using qualitative liquid chromtaography-mass spectrography.

Her presentation is suggestive of drug-induced vasculitis, likely secondary to levamisole-adulterated cocaine, complicated by an abscess. We started intravenous (IV) vancomycin and performed a bedside incision and drainage for the abscess on her posterior thigh. On the third day of hospital admission, all her lesions improved remarkably with abstinence from cocaine.

Levamisole, an atihelminthic agent, is used as treatment for autoimmune disorders and cancer due to its immunomodulating effects. Association between levamisole and cutaneous vasculitis was first described in 1978 in a patient who has rheumatoid arthritis treated with levamisole (1). (Macfarlane, 1978 #19) In 2000, the Food and Drug Administration (FDA) banned the use of levamisole due to its side effects profile.  However, since 2009, reports of agranulocytosis and vasculitis associated with levamisole have been increasing. The emergence of these cases is attributed by the increased contamination of cocaine with levamisole. More than 70% of cocaine found in North America contains levamisole (2,3).

Clinical features of levamisole-induced vasculitis include retiform purpura or purpura that has an angulated or branched configuration. Some patients may develop bacterial superinfection of the purpuric lesions, which was seen in our patient. This requires more attention to prevent further complications as these patients are immune suppressed. Two classic pathologic findings of these rashes are leukocytoclastic vasculitis of small vessels with fibrinoid necrosis of the wall of the vessels and formation of fibrin thrombi in the small vessels of superficial and deep dermis (4). These individuals commonly have neutropenia, positive ANCA serology, and elevated anti-MPO antibody titers (5).

Treatment includes supportive measures and abstinence from cocaine. Due to its increasing incidence, physician should be made aware of this disease entity due to its life threatening complications – neutropenia.

Kai Rou Tey, MD¹; Enrique Villavicencio, MD²; and Don Leo Pepito, MD¹

¹Department of Internal Medicine,

²Department of Neurology

University of Arizona College of Medicine-South Campus

Tucson, AZ

References

1. Macfarlane DG, Bacon PA. Levamisole-induced vasculitis due to circulating immune complexes. Br Med J. 1978 Feb 18;1(6110):407-8. [CrossRef] [PubMed]
2. Centers for Disease Control and Prevention (CDC). Agranulocytosis associated with cocaine use - four States, March 2008-November 2009. MMWR Morb Mortal Wkly Rep. 2009 Dec 18;58(49):1381-5. [CrossRef] [PubMed]
3. Buchanan JA, Heard K, Burbach C, Wilson ML, Dart R. Prevalence of levamisole in urine toxicology screens positive for cocaine in an inner-city hospital. JAMA. 2011 Apr 27;305(16):1657-8. [CrossRef] [PubMed]
4. Roberts JA, Chévez-Barrios P. Levamisole-Induced Vasculitis: A characteristic cutaneous vasculitis associated with levamisole-adulterated cocaine. Arch Pathol Lab Med. 2015 Aug;139(8):1058-61. [CrossRef] [PubMed]
5. McGrath MM, Isakova T, Rennke HG, Mottola AM, Laliberte KA, Niles JL. Contaminated cocaine and antineutrophil cytoplasmic antibody-associated disease. Clin J Am Soc Nephrol. 2011 Dec;6(12):2799-805. [CrossRef] [PubMed] 

Cite as: Tey KR, Villavicencio E, Pepito DL. Medical image of the week: levamisole-induced vasclitis. Southwest J Pulm Crit Care. 2016 Mar;12(4):149-51. doi: http://dx.doi.org/10.13175/swjpcc013-16 PDF