Polyarteritis Nodosa: A Systematic Review of Test Accuracy and Benefits and Harms of Common Treatments

Yih Chang Lin; Mohamad A Kalot; Nedaa M Husainat; Kevin Byram; Anisha B Dua; Karen E James; Jason M Springer; Marat Turgunbaev; Alexandra Villa-Forte; Andy Abril; Carol Langford; Mehrdad Maz; Sharon A Chung; Reem A Mustafa

doi:10.1002/acr2.11189

Polyarteritis Nodosa: A Systematic Review of Test Accuracy and Benefits and Harms of Common Treatments

ACR Open Rheumatol. 2021 Feb;3(2):91-100. doi: 10.1002/acr2.11189. Epub 2021 Jan 29.

Authors

Yih Chang Lin¹, Mohamad A Kalot², Nedaa M Husainat³, Kevin Byram⁴, Anisha B Dua⁵, Karen E James⁶, Jason M Springer⁴, Marat Turgunbaev⁷, Alexandra Villa-Forte⁸, Andy Abril⁹, Carol Langford⁸, Mehrdad Maz¹⁰, Sharon A Chung¹¹, Reem A Mustafa^{10

12}

Affiliations

¹ University of South Florida, Tampa, Florida, United States.
² The State University of New York at Buffalo, New York, United States.
³ St. Mary's Hospital, St. Louis, Missouri, United States.
⁴ Vanderbilt University Medical Center, Nashville, Tennessee, United States.
⁵ Northwestern University Feinberg School of Medicine, Chicago, Illinois, United States.
⁶ University of Utah Health, Salt Lake City, United States.
⁷ American College of Rheumatology, Atlanta, Georgia, United States.
⁸ Cleveland Clinic, Cleveland, Ohio, United States.
⁹ Mayo Clinic, Jacksonville, Florida, United States.
¹⁰ University of Kansas Medical Center, Kansas City, United States.
¹¹ University of California, San Francisco Medical Center, California, United States.
¹² Department of Health Research Methods, Evidence and Impact, McMaster University, Hamilton, Canada.

Abstract

Objective: The object of this study was to analyze the benefits and harms of different treatment options and to analyze test accuracy used in the evaluation of patients with primary systemic polyarteritis nodosa (PAN).

Methods: A systematic search of published English-language literature was performed in Ovid Medline, PubMed, Embase, and the Cochrane Library from the inception of each database through August 2019. Articles were screened for suitability in addressing patient, intervention, comparison, and outcome questions, with studies presenting the highest level of evidence given preference.

Results: Of 137 articles selected for data abstraction, we analyzed 21 observational studies and seven randomized controlled trials (RCTs). The results showed indirect evidence that a deep skin biopsy provides good diagnostic accuracy. A combined nerve and muscle biopsy should be obtained for patients with PAN with peripheral neuropathy. Cyclophosphamide with high-dose glucocorticoids (GCs) is effective as an induction treatment for newly diagnosed active and severe PAN. GC monotherapy is adequate in the majority of patients with nonsevere PAN, although it has a high relapse rate with GC taper. There was insufficient data in determining the optimal duration of non-GC and GC maintenance therapy. Tumor necrosis factor inhibitors are effective treatment for patients with deficiency of adenosine deaminase 2 (DADA2) with stroke and vasculitis manifestations.

Conclusion: This comprehensive systematic review synthesizes and evaluates the harms and benefits of different treatment options and the accuracy of commonly used tests for the diagnosis of systemic PAN. Data for diagnosis and management of PAN and DADA2 are mostly limited to observational studies. More high-quality RCTs are needed.