Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2023 Jun 16;59(6):1162.
doi: 10.3390/medicina59061162.

The "Viral" Form of Polyarteritis Nodosa (PAN)-A Distinct Entity: A Case Based Review

Affiliations
Review

The "Viral" Form of Polyarteritis Nodosa (PAN)-A Distinct Entity: A Case Based Review

Victoria Pașa et al. Medicina (Kaunas). .

Abstract

Classic polyarteritis nodosa (PAN) is a vasculitis with systemic manifestations that is characterized by inflammatory and necrotizing lesions affecting medium and small muscular arteries, most frequently at the bifurcation of the vessels. These lesions lead to the formation of microaneurysms, hemorrhaging ruptured aneurysms, thrombosis, and, consequently, ischemia or organ infarction. Background and Objectives: We present a complex clinical case of a patient with a late diagnosis of polyarteritis nodosa with multiorgan involvement. Materials and Methods: The 44-year-old patient, in an urban environment, presented on her own in the emergency room for acute ischemia phenomena and forearm and right-hand compartment syndrome, requiring surgical decompression in the Plastic Surgery Clinic. Results: Significant inflammatory syndrome is noted, alongside severe normocytic hypochromic iron deficiency anemia, nitrogen retention syndrome, hyperkalemia, hepatic syndrome, and immunological disturbances: absence of cANCA, pANCA, anti Scl 70 Ac, antinuclear Ac, and anti dDNA Ac, as well as a low C3 fraction of the plasmatic complement system. The morphological aspect described in the right-hand skin biopsy correlated with the clinical data supports the diagnosis of PAN. Conclusions: The viral form of PAN seems to be individualized as a distinct entity, requiring early, aggressive medication.

Keywords: inflammation; multisystem complications; polyarteritis nodosa (PAN); systemic vasculitis.

PubMed Disclaimer

Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. The sponsors had no role in the design, execution, interpretation, or writing of the study.

Figures

Figure 1
Figure 1
(a) Acute ischemia phenomena and forearm and right-hand compartment syndrome. (b) Bilateral hand onycholysis.
Figure 2
Figure 2
Skin biopsy (optical microscopy. Staining—hematoxylin-eosin): The dermis shows fibrosis. At the level of the hypodermis, a muscle-type artery with transmural polymorphic inflammatory infiltrate is identified, the lumen being obliterated by a recent fibrin-hematic thrombus. Fragmentation of the elastic limit.
Figure 3
Figure 3
Skin biopsy (optical microscopy. Staining—hematoxylin-eosin): The synovial fragment examined is covered with a slope of synoviocytes with preserved morphology. Underneath, marked edema, congestion, lymphocytic inflammatory infiltrate, and arterial-type vessels with lumen obliterated by recent thrombi are observed.
Figure 4
Figure 4
(a) Anterior chest X-ray showed the accentuation of the bilateral lung pattern and horizontal heart; (b) Angio CT highlights the extravasation of the contrast substance at the colonic level.
Figure 4
Figure 4
(a) Anterior chest X-ray showed the accentuation of the bilateral lung pattern and horizontal heart; (b) Angio CT highlights the extravasation of the contrast substance at the colonic level.
Figure 5
Figure 5
(ad) Dry gangrene at the level of the lower 2/3 of the left calf, bilateral leg and right hand, amputation of the left thigh is performed.
Figure 5
Figure 5
(ad) Dry gangrene at the level of the lower 2/3 of the left calf, bilateral leg and right hand, amputation of the left thigh is performed.

References

    1. Stanton M., Tiwari V. StatPearls. StatPearls Publishing; Treasure Island, FL, USA: 2023. [(accessed on 4 April 2023)]. Polyarteritis Nodosa. Available online: https://www.ncbi.nlm.nih.gov/books/NBK482157/ - PubMed
    1. Ozen S. The changing face of polyarteritis nodosa and necrotizing vasculitis. Nat. Rev. Rheumatol. 2017;6:381–386. doi: 10.1038/nrrheum.2017.68. - DOI - PubMed
    1. De Boysson H., Guillevin L. Polyarteritis Nodosa Neurologic Manifestations. Neurol. Clin. 2019;37:345–357. doi: 10.1016/j.ncl.2019.01.007. - DOI - PubMed
    1. Hernández-Rodríguez J., Alba M.A., Prieto-González S., Cid M.C. Diagnosis and classification of polyarteritis nodosa. J. Autoimmun. 2014;48–49:84–89. doi: 10.1016/j.jaut.2014.01.029. - DOI - PubMed
    1. Ramos-Casals M., Stone J.H., Cid M.C., Bosch X. The cryoglobulinaemias. Lancet. 2012;379:348–360. doi: 10.1016/S0140-6736(11)60242-0. - DOI - PubMed

Substances