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Arthritis & Rheumatology (Hoboken, N.J.) Aug 2021To provide evidence-based recommendations and expert guidance for the management of systemic polyarteritis nodosa (PAN).
OBJECTIVE
To provide evidence-based recommendations and expert guidance for the management of systemic polyarteritis nodosa (PAN).
METHODS
Twenty-one clinical questions regarding diagnostic testing, treatment, and management were developed in the population, intervention, comparator, and outcome (PICO) format for systemic, non-hepatitis B-related PAN. Systematic literature reviews were conducted for each PICO question. The Grading of Recommendations Assessment, Development and Evaluation methodology was used to assess the quality of evidence and formulate recommendations. Each recommendation required ≥70% consensus among the Voting Panel.
RESULTS
We present 16 recommendations and 1 ungraded position statement for PAN. Most recommendations were graded as conditional due to the paucity of evidence. These recommendations support early treatment of severe PAN with cyclophosphamide and glucocorticoids, limiting toxicity through minimizing long-term exposure to both treatments, and the use of imaging and tissue biopsy for disease diagnosis. These recommendations endorse minimizing risk to the patient by using established therapy at disease onset and identify new areas where adjunctive therapy may be warranted.
CONCLUSION
These recommendations provide guidance regarding diagnostic strategies, use of pharmacologic agents, and imaging for patients with PAN.
Topics: Antirheumatic Agents; Cyclophosphamide; Disease Management; Evidence-Based Medicine; Glucocorticoids; Humans; Polyarteritis Nodosa; Rheumatology; United States
PubMed: 34235883
DOI: 10.1002/art.41776 -
Rheumatology International Aug 2008Polyarteritis nodosa (PAN) is not commonly associated with hematologic abnormalities. We report the first case of pancytopenia as the presenting symptom of PAN. There... (Review)
Review
Polyarteritis nodosa (PAN) is not commonly associated with hematologic abnormalities. We report the first case of pancytopenia as the presenting symptom of PAN. There was no associated malignancy or reactive hemophagocytic syndrome (RHS) to explain the findings. The patient, after successful treatment of PAN, subsequently developed RHS a few years later. He is the fourth reported case of RHS in a patient with PAN. In addition to describing the patient's presentation and clinical course, a systematic review of the literature was performed examining the association between hematologic conditions, including RHS, and PAN.
Topics: Adult; Humans; Male; Pancytopenia; Polyarteritis Nodosa
PubMed: 18368407
DOI: 10.1007/s00296-008-0572-1 -
Seminars in Arthritis and Rheumatism Dec 2021Deficiency of adenosine deaminase 2 (DADA2) is a rare autoinflammatory disease usually presenting before the age of 10 years. Non-specific clinical features or...
BACKGROUND
Deficiency of adenosine deaminase 2 (DADA2) is a rare autoinflammatory disease usually presenting before the age of 10 years. Non-specific clinical features or late-onset presentation may delay its diagnosis until adulthood.
OBJECTIVE
To determine whether DADA2 diagnosed in adulthood is associated with specific characteristics compared to DADA2 diagnosed in childhood.
METHODS
We pooled a cohort of 12 adult DADA2 patients followed in France with cases identified through a systematic literature review. For each patient, we determined the type of clinical presentation and assessed six key organ involvements.
RESULTS
A total of 306 cases were included. Among the 283 patients with available data regarding age at diagnosis, 140 were diagnosed during adulthood and 143 during childhood. The vascular presentation of DADA2 was more frequent in the adult diagnosis group (77.9% vs. 62.9%, p < 0.01), whereas the hematological presentation (bone marrow failure) prevailed in the pediatric diagnosis group (10.0% vs. 20.3% p = 0.02). In patients with vasculopathy, severe skin manifestations developed in 35% and 10% of the adult and pediatric diagnosis groups, respectively. Conversely, fewer strokes occurred in the adult group presenting with systemic vasculopathy (54% vs. 81%). Symptomatic humoral immune deficiency (HID) was rarely a clinical presentation in itself (5% and 2.8%) but accompanied other phenotypes of DADA2, especially the hematological phenotype in the adult group (33% vs. 4%).
CONCLUSION
DADA2 diagnosed in adulthood presents more often with a vascular phenotype and less often with bone marrow failure than DADA2 diagnosed in childhood. Adults diagnosed with DADA2 vasculopathy display more severe skin involvement but fewer strokes.
Topics: Adenosine Deaminase; Adult; Child; Humans; Immunologic Deficiency Syndromes; Intercellular Signaling Peptides and Proteins; Mutation; Phenotype
PubMed: 34571400
DOI: 10.1016/j.semarthrit.2021.09.001