Granulomatosis With Polyangiitis in Untreated Graves' Disease: A Rare Event

Abstract

Antithyroid medications can cause antineutrophil cytoplasmic antibody (ANCA) vasculitis. However, no literature in English describes the coexistence of granulomatosis with polyangiitis (GPA) and untreated Graves' disease. We present a 19-year-old female with thyroid storm and additional complaints of cough, hemoptysis, nasal discharges, polyarthralgia, and skin lesions. Imaging showed peri-hilar cavities and acute-on-chronic sinusitis. Elevated cytoplasmic pattern antineutrophil cytoplasmic antibody (C-ANCA) and anti-proteinase-3 antibody levels plus histopathology of the nasal and skin biopsies suggested GPA. Propranolol, methimazole, and potassium-iodide resolved the thyroid storm. Induction therapy (steroids, rituximab) for GPA provided relief of chronic symptoms stressing the importance of early recognition and swift initiation of treatment.

Keywords: anca associated vasculitis; coexistence; granulomatosis with polyangiitis (gpa); graves´disease; rituximab therapy; thyroid-storm.

Figure 1. A chest X-ray shows bilateral perihilar opacities without lymphadenopathy

Figure 2. The CT images of the chest (one slice shown above) without contrast show multiple cavities with thick and irregular walls with adjacent lung consolidation. No lymphadenopathy.

Figure 3. The CT images of the paranasal sinuses show right frontal (blue arrow), right ethmoid (yellow arrow), right maxillary (red arrow), and bilateral sphenoid acute-on-chronic sinusitis (green arrows).

Figure 4. Histopathology of the left nasal lateral wall biopsy specimen shows poorly defined granulomas with an admixture of acute and chronic inflammatory cells that include neutrophils, plasma cells, lymphocytes, histiocytes (Panel A), and a few eosinophils with multinucleated giant cells (Panel B). The histopathologist noted that no defined foreign body was identified, and examination with polarized light did not demonstrate birefringence.

Figure 5. Punched-out ulceration (approximately 3 mm in diameter) with minimal bloody drainage located on the right arm. Similar lesions were seen on the right side of the face, back, left arm, and left axilla.

Figure 6. Histopathology of punch biopsy of one of the ulcerated skin lesions on the upper back shows dermal infiltration of histiocytes, some multinucleated (Panel A), as well as lymphocytes, and plasma cells. There are extravasated erythrocytes and fibrin adjacent to blood vessels (Panel B). In the context of cavitary lung lesions, elevated C-ANCA and anti-PR3 antibodies, the histopathologists’ opinion was that the microscopic findings were consistent with granulomatosis with polyangiitis.

C-ANCA: Cytoplasmic pattern antineutrophil cytoplasmic antibodies, PR3: Proteinase-3