Granulomatosis with Polyangiitis (Wegener’s Granulomatosis) Co-Existing with Antiphospholipid Syndrome – An Unusual Cutaneous Presentation

Neha Rana; Humaira Talat; Maryam Rehan; Reema Mirza; Batool ur Rehman

doi:10.36283/PJMD13-3/026

Authors

Neha Rana Dow University of Health Sciences
Humaira Talat Dow University of Health Sciences
Maryam Rehan Dr. Ruth K.M Pfau Civil Hospital Karachi
Reema Mirza Dow University of Health Sciences
Batool ur Rehman Dow University of Health Sciences

DOI:

https://doi.org/10.36283/PJMD13-3/026

Abstract

Granulomatosis with polyangiitis (GPA) is an autoimmune disease that causes inflammation of the small blood vessels and involves many organs, most commonly the upper and lower respiratory tracts and the kidneys. GPA can also affect other organs, such as the eyes, skin, and nervous system. Meanwhile, Antiphospholipid syndrome (APS) is known to cause arterial or venous thrombosis in various organ systems. We report the case of a 54-year-old Pakistani man diagnosed with granulomatosis with polyangiitis co-existing with antiphospholipid syndrome. He presented with unusual cutaneous manifestations, positive c-ANCA, antiphospholipid antibodies, and distinctive renal histopathological features that helped rule out other potential differentials. The patient ultimately received treatment with a high dose of prednisolone, monthly pulses of cyclophosphamide, and anticoagulation, leading to significant improvement in both clinical and laboratory findings. This case underscores the complexity of autoimmune diseases and the importance of comprehensive evaluation for accurate diagnosis and management.

Keywords: Gangrene, Wegener’s Granulomatosis, Antiphospholipid Syndrome, Anti Neutrophil Cytoplasmic Antibodies, Antiphospholipid Antibodies.

Author Biography

Batool ur Rehman, Dow University of Health Sciences

Dermatology, Dow University of Health Sciences

References

Granulomatosis with Polyangiitis. Available from: https://www.ncbi.nlm.nih.gov/books/NBK557827/

Truglia S, Capozzi A, Mancuso S, Manganelli V, Rapino L, Riitano G, et al. Relationship Between Gender Differences and Clinical Outcome in Patients With the Antiphospholipid Syndrome. Front Immunol. 2022; 13:932181. doi: 10.3389/fimmu.2022.932181.

Montero-Vilchez T, Martinez-Lopez A, Salvador-Rodriguez L, Del Carmen Ramírez-Barberena M, Tercedor-Sanchez J, Molina-Leyva A, Arias-Santiago S. Cutaneous Manifestations of Granulomatosis with Polyangiitis: A Case Series Study. Acta Derm Venereol. 2020;100(10):adv00150. doi: 10.2340/00015555-3506.

Lau RA, Bains R, Suraweera D, Ma J, Heinze ER, Wong AL, Clements PJ. A Rare Case of Digital Ischemia and Gangrene in ANCA-Associated Vasculitis with Review of the Literature. Case Rep Rheumatol. 2017; 2017:2421760. doi: 10.1155/2017/2421760.

Miyakis S, Lockshin MD, Atsumi T, Branch DW, Brey RL, Cervera R, et al. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost. 2006;4(2):295-306. doi: 10.1111/j.1538-7836.2006.01753.x.

Jari M. Thrombosis as the First Manifestation of Granulomatosis with Polyangiitis Disease in an Adolescent. Case Rep Hematol. 2021; 2021:5520258. doi: 10.1155/2021/5520258.

Yoo J, Ahn SS, Jung SM, Song JJ, Park YB, Lee SW. Persistent antiphospholipid antibodies are associated with thrombotic events in ANCA-associated vasculitis: A retrospective monocentric study. Nefrologia (Engl Ed). 2019;39(4):395-401. doi: 10.1016/j.nefro.2018.10.014.

Sebastian JK, Voetsch B, Stone JH, Romay-Penabad Z, Lo GH, Allen NB, et al.; Wegener's Granulomatosis Etanercept Trial Research Group. The frequency of anticardiolipin antibodies and genetic mutations associated with hypercoagulability among patients with Wegener's granulomatosis with and without a history of a thrombotic event. J Rheumatol. 2007;34(12):2446-2450.

Akhlaghi Kalahroodi M, Loghman M, Ramezanpoor M, Shahriarirad R, Rahmanian E. Antineutrophil cytoplasmic antibody-associated vasculitis in presence of positive antiphospholipid antibody: a case report. J Med Case Rep. 2022;16(1):28. doi: 10.1186/s13256-022-03256-3.

Meroni PL, Del Papa N, Beltrami B, Tincani A, Balestrieri G, Krilis SA. Modulation of endothelial cell function by antiphospholipid antibodies. Lupus. 1996;5(5):448-450. doi: 10.1177/096120339600500523.