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 Table of Contents  
LETTERS TO EDITOR
Year : 2022  |  Volume : 17  |  Issue : 2  |  Page : 210-212

Granulomatosis with polyangiitis and COVID-19 pneumonia


1 Department of Rheumatology, Southport and Ormskirk Hospital NHS Trust, Southport, United Kingdom
2 Department of Orthopaedics, Southport and Ormskirk NHS Trust, Southport, United Kingdom
3 Department of Rheumatology, The Royal Wolverhampton NHS Trust, Wolverhampton, United Kingdom
4 Department of Pulmonary and Critical Care Medicine, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India
5 Department of Medicine, Southport and Ormskirk NHS Trust, Southport, United Kingdom

Date of Submission11-Oct-2021
Date of Acceptance07-Nov-2021
Date of Web Publication22-Jan-2022

Correspondence Address:
Dr. Arvind Nune
Department of Rheumatology, Southport and Ormskirk Hospital NHS Trust, Southport
United Kingdom
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/injr.injr_235_21

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How to cite this article:
Nune A, Iyengar KP, Mulherin D, Ish P, Musat C A, Sapkota HR. Granulomatosis with polyangiitis and COVID-19 pneumonia. Indian J Rheumatol 2022;17:210-2

How to cite this URL:
Nune A, Iyengar KP, Mulherin D, Ish P, Musat C A, Sapkota HR. Granulomatosis with polyangiitis and COVID-19 pneumonia. Indian J Rheumatol [serial online] 2022 [cited 2022 Jul 4];17:210-2. Available from: https://www.indianjrheumatol.com/text.asp?2022/17/2/210/336274



Dear Editor,

Granulomatosis with polyangiitis (GPA), a form of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), is a rare autoimmune condition with a poor prognosis if not diagnosed and treated promptly with appropriate immunosuppression. GPA and COVID-19 share several clinical and laboratory features: Breathlessness, kidney injury, skin rashes, ground-glass lung changes, and elevated inflammatory markers. Greater attention has been given to the management of COVID-19 during the pandemic, particularly its complications during hospital admissions. As a result, timely delivery of elective and emergency care for systemic autoimmune rheumatic disorders (SARD) have been affected.[1] This has raised concerns about the management of AAV during the COVID-19 pandemic, as AAV may present with nonspecific symptoms and typically has systemic involvement, as seen in this case.[2]

A 71-year-old Caucasian gentleman was admitted with a 6-week history of productive cough, hemoptysis, breathlessness, weight loss, night sweats, otalgia, and hearing loss. He also reported a loss of smell and taste. On admission, his oxygen saturations were 97% on 2 l/min oxygen, and he had a fever of 38°C. The patient tested positive for SARS-CoV-2 by Reverse Transcriptase Polymerase Chain Reaction (RT-PCR) test from a nasopharyngeal swab. His C-reactive protein was elevated 173 mg/L (normal range 0–5), and his white cell count of 11.1 × 109/L (3.7–9.5). He had a serum creatinine of 225 μmol/L (45–84) and an estimated glomerular filtration rate (GFR) of 24 ml/min/1.73 m2 (normal range >60). Blood and sputum cultures were negative for bacterial infection and tuberculosis. Urine analysis detected protein and red blood cells casts. His chest radiograph [Figure 1]a on admission showed bilateral pulmonary ground-glass opacities (GGOs). The medical team diagnosed the patient with COVID-19 pneumonia, and he was managed with supplemental oxygen, antibiotics, and oral dexamethasone 6 mg daily for 10 days. As the patient's dyspnea worsened, a repeat chest radiograph [Figure 1]b showed worsening of ground-glass changes, and repeat bloods revealed deterioration of kidney function (serum creatinine measured 442 μmol/L and eGFR 10 ml/min/1.73 m2). Further immunology workup confirmed positive proteinase 3-antineutrophil cytoplasmic antibodies (PR3-ANCA) quantified at 7.9 units (0–1.9). Therefore, a rheumatology review was sought. Rheumatology review on day 11 postadmission identified a purpuric rash on his toes, soles of feet [Figure 1]d and [Figure 1]e, and fingers [Figure 1]f. A computed tomography scan [Figure 1]c of the thorax confirmed bilateral pulmonary GGOs along with right basal pleural effusion. A bronchoscopy revealed blood in the bronchial tree, but bronchial washings confirmed no malignancy or acid-fast bacilli.
Figure 1: Chest X-ray of the patient at presentation showing bilateral ground-glass opacities (a) worsening during hospitalization (b); computed tomography chest image showing bilateral ground-glass shadows with right pleural effusion (c); purpuric rash on his toes, soles of feet (d and e); and fingers (f)

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Based on the above results, including the positive PR3-ANCA, a concurrent diagnosis of GPA was made. On day 15, he was commenced intravenous (IV) methylprednisolone, 1 g daily for 3 days, followed by oral prednisolone 60 mg a day. He was subsequently commenced on oral cyclophosphamide. The patient made a slow but steady recovery and was discharged 20 days later, after a 35-day hospital stay.

COVID toes typically present with red or purple discoloration and swelling mainly in the toes and fingers, which may lead to blisters. Even though the exact etiology is unknown, the possible underlying mechanisms include immune dysregulation causing the release of interferon-1 and/or coagulation abnormality. They are usually painless and do not involve the proximal parts of the limbs.[3] Purpuric skin rashes in hands and feet are, however, common skin manifestations in GPA and supported the diagnosis in our patient.[4] Similarly, pulmonary infiltrates, the most common lung manifestations in patients with COVID-19 pneumonia, are also seen in patients with GPA. Bronchoscopy evidence of bronchial wall erythema and bleeding suggests underlying alveolar hemorrhage as hemoptysis is not common in COVID-19 pneumonia. Peripheral pulmonary GGO is often seen in COVID-19 pneumonia; however, pleural effusion is uncommon.[5] Although radiological findings of GPA are widely variable, subpleural cavitation and opacities are also reported in GPA. Diffuse pulmonary opacities in GPA can be associated with pulmonary hemorrhage and hemoptysis.

The reported case had acute kidney injury (AKI), which has also been reported in patients with COVID-19 infection.[6] However, a week after the patient received IV methylprednisolone, he no longer required oxygen support, and his nephropathy recovered. Rapid improvement of the patient's clinical condition with IV methylprednisolone emphasizes that GPA was a likely cause of his AKI. Similarly, false-positive ANCA can be seen in COVID-19 due to immune-mediated mechanisms.[7] However, relatively high PR3-ANCA titer (6.2 units) and a clinical collaboration helped differentiate from an underlying immune vasculitis.

SARD patients on long-term corticosteroids and multiple comorbidities are at an increased risk of contracting COVID-19.[8] There are little literature data on the choice of immunosuppressive drug in AAV with COVID-19. Rituximab has a long half-life and risk of severe COVID-19 pneumonia and bacterial infections.[9] Even though pulse cyclophosphamide is the preferred choice for AAV remission induction therapy, we chose oral cyclophosphamide in our case to minimize the patient's hospital visits during the pandemic. It is recommended to withhold immunosuppressive drugs in patients with SARD while treating COVID-19 until the infection is cleared; however, it is prudent to give immunosuppressive drugs to treat life or organ-threatening manifestations of GPA with caution, even with COVID-19 infection.[10]

To conclude, GPA presenting simultaneously with COVID-19 pneumonia can lead to an initial diagnostic and therapeutic dilemma. A high index of suspicion, clinical evaluation, and laboratory investigations can help arrive at an early diagnosis so that appropriate early treatment, including immunosuppression, can be initiated.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that their name and initial will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Nune A, Iyengar KP, Ahmed A, Bilgrami S, Sapkota HR. Impact of COVID-19 on rheumatology practice in the UK – A pan-regional rheumatology survey. Clin Rheumatol 2021;40:2499-504.  Back to cited text no. 1
    
2.
Kant S, Morris A, Ravi S, Floyd L, Gapud E, Antichos B, et al. The impact of COVID-19 pandemic on patients with ANCA associated vasculitis. J Nephrol 2021;34:185-90.  Back to cited text no. 2
    
3.
Galván Casas C, Català A, Carretero Hernández G, Rodríguez-Jiménez P, Fernández-Nieto D, Rodríguez-Villa Lario A, et al. Classification of the cutaneous manifestations of COVID-19: A rapid prospective nationwide consensus study in Spain with 375 cases. Br J Dermatol 2020;183:71-7.  Back to cited text no. 3
    
4.
Francès C, Du LT, Piette JC, Saada V, Boisnic S, Wechsler B, et al. Wegener's granulomatosis. Dermatological manifestations in 75 cases with clinicopathologic correlation. Arch Dermatol 1994;130:861-7.  Back to cited text no. 4
    
5.
Gianfrancesco M, Hyrich KL, Al-Adely S, Carmona L, Danila MI, Gossec L, et al. Characteristics associated with hospitalisation for COVID-19 in people with rheumatic disease: Data from the COVID-19 global rheumatology alliance physician-reported registry. Ann Rheum Dis 2020;79:859-66.  Back to cited text no. 5
    
6.
Nadim MK, Forni LG, Mehta RL, Connor MJ Jr., Liu KD, Ostermann M, et al. COVID-19-associated acute kidney injury: Consensus report of the 25th Acute Disease Quality Initiative (ADQI) Workgroup. Nat Rev Nephrol 2020;16:747-64.  Back to cited text no. 6
    
7.
Bornstein G, Ben-Zvi I, Furie N, Grossman C. Clinical significance of positive anti-neutrophil cytoplasmic antibodies without evidence of anti-neutrophil cytoplasmic antibodies-associated vasculitis. Int J Rheum Dis 2019;22:940-5.  Back to cited text no. 7
    
8.
Bachiller-Corral J, Boteanu A, Garcia-Villanueva MJ, de la Puente C, Revenga M, Diaz-Miguel MC, et al. Risk of severe COVID-19 infection in patients with inflammatory rheumatic diseases. J Rheumatol 2021;48:1098-102.  Back to cited text no. 8
    
9.
Guilpain P, Le Bihan C, Foulongne V, Taourel P, Pansu N, Maria AT, et al. Rituximab for granulomatosis with polyangiitis in the pandemic of COVID-19: Lessons from a case with severe pneumonia. Ann Rheum Dis 2021;80:e10.  Back to cited text no. 9
    
10.
National Institute for Health and Care Excellence. COVID-19 Rapid Guideline: Rheumatological Autoimmune, Inflammatory and Metabolic Bone Disorders. NICE Guideline [NG167]; 2020. Available from: https://www.nice.org.uk/guidance/ng167/chapter/3-Patients-known-or-suspected-to-have-COVID-19. [Last accessed on 2021 May 30].  Back to cited text no. 10
    


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