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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sahar Memar Montazerin, M.D.[2]

Synonyms and Keywords: PAPA syndrome

Overview

Pyogenic sterile arthritis, pyoderma gangrenosum, acne syndrome, PAPA syndrome, is one of the inherited autoinflammatory disorders. PAPA syndrome presents with the triad of aseptic arthritis, pyoderma gangrenosum, and acne. However, not all the features may be present in all the patients.

Historical Perspective

  • PAPA syndrome was first discovered by Dr. Noralane M. Lindor, in 1997 following visiting several patients from three generations of a family with similar presentations.[1]
  • The association between PSTPIP1 gene mutation and PAPA syndrome was made in the year 2000.[2]

Classification

  • There is no established system for the classification of PAPA syndrome.

Pathophysiology

Causes

Differentiating PAPA syndrome from Other Diseases

Epidemiology and Demographics

  • The prevalence of PAPA syndrome is approximately 0.01 case per 100,000 individuals worldwide.[5]
  • There are 53 cases of PAPA syndrome in the literature.[6]
  • PAPA syndrome commonly affects children. However, it may develop later in some individuals.
  • There is no racial predilection to PAPA syndrome.
  • PAPA syndrome affects men and women equally.
  • The majority of PAPA syndrome cases are reported in Europe, New Zealand, and the USA.

Risk Factors

Screening

  • There is insufficient evidence to recommend routine screening for PAPA syndrome.

Natural History, Complications, and Prognosis

Diagnosis

Diagnostic Study of Choice

History and Symptoms

  • A positive history of recurrent arthritis, skin ulceration, and acne is suggestive of PAPA syndrome.[5]
  • The presenting symptom of PAPA syndrome is usually culture-negative arthritis.

Physical Examination

Laboratory Findings

Electrocardiogram

X-ray

Echocardiography or Ultrasound

CT scan

MRI

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

References

  1. Lindor, Noralane M.; Arsenault, Todd M.; Solomon, Herman; Seidman, Christine E.; McEvoy, Marian T. (1997). "A New Autosomal Dominant Disorder of Pyogenic Sterile Arthritis, Pyoderma Gangrenosum, and Acne: PAPA Syndrome". Mayo Clinic Proceedings. 72 (7): 611–615. doi:10.4065/72.7.611. ISSN 0025-6196.
  2. 2.0 2.1 Yeon, Howard B.; Lindor, Noralane M.; Seidman, J.G.; Seidman, Christine E. (2000). "Pyogenic Arthritis, Pyoderma Gangrenosum, and Acne Syndrome Maps to Chromosome 15q". The American Journal of Human Genetics. 66 (4): 1443–1448. doi:10.1086/302866. ISSN 0002-9297.
  3. J. Smith, Elisabeth; Allantaz, Florence; Bennett, Lynda; Zhang, Dongping; Gao, Xiaochong; Wood, Geryl; L. Kastner, Daniel; Punaro, Marilynn; Aksentijevich, Ivona; Pascual, Virginia; A. Wise, Carol (2010). "Clinical, Molecular, and Genetic Characteristics of PAPA Syndrome: A Review". Current Genomics. 11 (7): 519–527. doi:10.2174/138920210793175921. ISSN 1389-2029.
  4. Shoham, N. G.; Centola, M.; Mansfield, E.; Hull, K. M.; Wood, G.; Wise, C. A.; Kastner, D. L. (2003). "Pyrin binds the PSTPIP1/CD2BP1 protein, defining familial Mediterranean fever and PAPA syndrome as disorders in the same pathway". Proceedings of the National Academy of Sciences. 100 (23): 13501–13506. doi:10.1073/pnas.2135380100. ISSN 0027-8424.
  5. 5.0 5.1 5.2 Schellevis, M. A.; Stoffels, M.; Hoppenreijs, E. P. A. H.; Bodar, E.; Simon, A.; van der Meer, J. W. M. (2011). "Variable expression and treatment of PAPA syndrome". Annals of the Rheumatic Diseases. 70 (6): 1168–1170. doi:10.1136/ard.2009.126185. ISSN 0003-4967.
  6. 6.0 6.1 6.2 6.3 Martinez-Rios, Claudia; Jariwala, Mehul P.; Highmore, Kerri; Duffy, Karen Watanabe; Spiegel, Lynn; Laxer, Ronald M.; Stimec, Jennifer (2018). "Imaging findings of sterile pyogenic arthritis, pyoderma gangrenosum and acne (PAPA) syndrome: differential diagnosis and review of the literature". Pediatric Radiology. 49 (1): 23–36. doi:10.1007/s00247-018-4246-1. ISSN 0301-0449.
  7. 7.0 7.1 7.2 7.3 Dierselhuis, M. P. (2005). "Anakinra for flares of pyogenic arthritis in PAPA syndrome". Rheumatology. 44 (3): 406–408. doi:10.1093/rheumatology/keh479. ISSN 1460-2172.
  8. Horiuchi, Isao; Fukatsu, Yuko; Ushijima, Junko; Nakamura, Eishin; Samajima, Koki; Kadowaki, Kanako; Takagi, Kenjiro (2016). "A pregnancy-associated nonfamilial case of PAPA (pyogenic sterile arthritis, pyoderma gangrenosum, acne) syndrome". Clinical Case Reports. 4 (10): 989–991. doi:10.1002/ccr3.662. ISSN 2050-0904.
  9. Hong, Jin-Bon; Su, Yi-Ning; Chiu, Hsien-Ching (2009). "Pyogenic arthritis, pyoderma gangrenosum, and acne syndrome (PAPA syndrome): Report of a sporadic case without an identifiable mutation in the CD2BP1 gene". Journal of the American Academy of Dermatology. 61 (3): 533–535. doi:10.1016/j.jaad.2008.11.017. ISSN 0190-9622.
  10. Cortis, Elisabetta; De Benedetti, Fabrizio; Insalaco, Antonella; Cioschi, Stefania; Muratori, Flaminia; D'Urbano, Leila E.; Ugazio, Alberto G. (2004). "Abnormal production of the tumor necrosis factor inhibitor etanercept and clinical efficacy of tumor in a patient with PAPA syndrome". The Journal of Pediatrics. 145 (6): 851–855. doi:10.1016/j.jpeds.2004.08.001. ISSN 0022-3476.
  11. Tallon, B.; Corkill, M. (2006). "Peculiarities of PAPA syndrome". Rheumatology. 45 (9): 1140–1143. doi:10.1093/rheumatology/kei178. ISSN 1462-0332.
  12. Demidowich, Andrew P.; Freeman, Alexandra F.; Kuhns, Douglas B.; Aksentijevich, Ivona; Gallin, John I.; Turner, Maria L.; Kastner, Daniel L.; Holland, Steven M. (2012). "Brief Report: Genotype, phenotype, and clinical course in five patients with PAPA syndrome (pyogenic sterile arthritis, pyoderma gangrenosum, and acne)". Arthritis & Rheumatism. 64 (6): 2022–2027. doi:10.1002/art.34332. ISSN 0004-3591.



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