title: Repository corticotropin injection in a patient presenting with focal segmental glomerulosclerosis, rheumatoid arthritis, and optic neuritis: a case report.
abstract: BACKGROUND
  Focal segmental glomerulosclerosis (FSGS) causes scarring or sclerosis of glomeruli that act as tiny filters in the kidneys, damage to which results in diminished ability to properly filter blood, resulting in the urinary loss of plasma proteins and subsequent proteinuria.
  
  
  METHODS
  A 60-year-old, white female with a history of intermittent proteinuria was referred by her primary care physician for renal dysfunction. Biopsy confirmed FSGS and she was treated with an angiotensin-converting enzyme inhibitor. She also had rheumatoid arthritis (RA) but no active synovitis and was maintained on prednisone 5 mg/d. She also complained of worsening vision in her right eye and was diagnosed with optic neuritis (ON). She remained stable for about 8 months when examination indicated FSGS relapse, and she reported painful RA flares. She was treated with Acthar(®) Gel (40 mg biweekly) for 6 months, after which proteinuria and urine protein-to-creatinine ratio decreased to about half. Her ON improved, and she reported that she had fewer RA flares and pain improved by 50%. This case of confirmed FSGS showed an improved response to treatment with Acthar Gel for FSGS with concomitant RA and ON.
  
  
  CONCLUSIONS
  This referral case is relevant to primary care practitioners who treat disorders that may be responsive to corticosteroid therapy. The antiproteinuric effects and ancillary improvement in RA and ON symptoms during treatment with Acthar Gel are not entirely explained by its steroidogenic actions. ACTH is a bioactive peptide that, together with α-melanocyte-stimulating hormone, exhibits biologic efficacy by modulating proinflammatory cytokines and subsequent leukocyte extravasation and may have autocrine/paracrine effects in joints. While Acthar Gel was primarily administered in this case to treat proteinuria, it also showed ancillary benefits in patients with concomitant inflammatory disease states.
authors.affiliation: Nephrology Associates of Central Florida, PA, Orlando, FL, USA.
authors.forename: Arvind
authors.initials: A
authors.lastname: Madan
keywords: Acthar Gel; adrenocorticotropic hormone; nephrotic syndrome; proteinuria
references.citation: FASEB J. 2010 Dec;24(12):4835-43
references.pmid: 20702773
references.citation: Curr Opin Rheumatol. 2013 May;25(3):375-83
references.pmid: 23508130
references.citation: N Engl J Med. 2006 Mar 23;354(12):1273-80
references.pmid: 16554529
references.citation: J Neurol. 2000 Jun;247(6):435-42
references.pmid: 10929272
references.citation: Pharmacol Ther. 2006 Jul;111(1):1-15
references.pmid: 16488018
references.citation: J Am Soc Nephrol. 2010 Aug;21(8):1290-8
references.pmid: 20507942
references.citation: N Engl J Med. 2009 Jan 1;360(1):44-52
references.pmid: 19092146
references.citation: Arthritis Rheum. 2010 Sep;62(9):2569-81
references.pmid: 20872595
references.citation: Pharmacogenomics. 2013 Mar;14(4):425-44
references.pmid: 23438889
references.citation: Kidney Int. 2008 Feb;73(4):399-406
references.pmid: 17989648
references.citation: Pharmacol Rev. 2004 Mar;56(1):1-29
references.pmid: 15001661
references.citation: Drug Des Devel Ther. 2011 Mar 14;5:147-53
references.pmid: 21448451
references.citation: Adv Chronic Kidney Dis. 2014 Mar;21(2):134-51
references.pmid: 24602463
references.citation: Curr Opin Nephrol Hypertens. 2007 Jan;16(1):32-8
references.pmid: 17143069
references.citation: Am J Nephrol. 2012;36(1):58-67
references.pmid: 22722778
references.citation: Med Health R I. 2008 Feb;91(2):57-9
references.pmid: 18372861
references.citation: Clin J Am Soc Nephrol. 2013 Dec;8(12):2072-81
references.pmid: 24009220
references.citation: Control Clin Trials. 1991 Aug;12(4):486-503
references.pmid: 1657526
references.citation: ScientificWorldJournal. 2009 Dec 16;9:1394-414
references.pmid: 20024514
references.citation: Nat Rev Nephrol. 2011 Dec 06;8(2):122-8
references.pmid: 22143333