Man, 31 years old

Image not of actual patient.

Vogt-Koyanagi-Harada disease

Man, aged 31 years, with Vogt-Koyanagi-Harada (VKH) disease*

Initial presentation1:

  • VA 20/200 OD, 20/30 OS
  • Full clinical exam and IVFA imaging confirmed VKH diagnosis

*This case is based on a single patient and is not representative of the overall patient population. Reimbursement for Acthar Gel may differ based on the patient’s health insurance plan.

Treatment history1

Treatment Outcome

Oral corticosteroids

1 week after beginning treatment:

  • Subretinal fluid and anterior cell reaction worsened
  • VA decreased to HM OD and CF at 4 feet OS
  • Patient required an alternative treatment regimen
  • Multiple topical corticosteroids
  • Periocular injection OU of triamcinolone acetonide

5 weeks after initial presentation:

  • Subretinal fluid decreased
  • VA improved to 20/60+2 OD and 20/40 OS

6 to 8 months after initial presentation:

  • Trace number of cells in the vitreous and uveitis/choroiditis present
  • VKH still evident OU
  • Subretinal fluid resolved

Treatment

Oral corticosteroids

Outcome

1 week after beginning treatment:

  • Subretinal fluid and anterior cell reaction worsened
  • VA decreased to HM OD and CF at 4 feet OS
  • Patient required an alternative treatment regimen

Treatment

  • Multiple topical corticosteroids
  • Periocular injection OU of triamcinolone acetonide

Outcome

5 weeks after initial presentation:

  • Subretinal fluid decreased
  • VA improved to 20/60+2 OD and 20/40 OS
 

6 to 8 months after initial presentation:

  • Trace number of cells in the vitreous and uveitis/choroiditis present
  • VKH still evident OU
  • Subretinal fluid resolved

Before treatment with Acthar Gel. Fundus (A) and FA (B) of OD. Note the multiple hyperfluorescent foci and dye pooling.

1 year of treatment with Acthar Gel1

  • Acthar Gel subcutaneous injection 80 U twice a week
  • Oral NSAID (ketorolac)
  • Topical prednisolone (Pred Forte®)
  • Oral prednisone
Acthar Gel subcutaneous injection 40 U twice a week

7 months after Acthar Gel initiation:

  • FA and ICG imaging showed no leakage or subretinal fluid
  • Acthar Gel was reduced to 40 U twice a week

1 year after Acthar Gel initiation:

  • No signs of leakage, bleeding, or edema
  • No side effects reported
  • Patient planned to continue treatment with Acthar Gel
  • Acthar subcutaneous injection 80 U twice a week
  • Oral NSAID (ketorolac)
  • Topical prednisolone (Pred Forte®)
  • Oral prednisone

7 months after Acthar Gel initiation:

  • FA and ICG imaging showed no leakage or subretinal fluid
  • Acthar Gel was reduced to 40 U twice a week

Acthar Gel subcutaneous injection 40 U twice a week

1 year after Acthar Gel initiation:

  • No signs of leakage, bleeding, or edema
  • No side effects reported
  • Patient planned to continue treatment with Acthar Gel

Common adverse reactions for Acthar Gel are similar to those of corticosteroids and include fluid retention, alteration in glucose tolerance, elevation in blood pressure, behavioral and mood changes, increased appetite, and weight gain.

After 7 months of treatment with Acthar Gel. Fundus (A) and FA (B) of OD. A similar appearance was seen OS.

Case provided by:

Kent Small, MD, and Fadi Shaya, CRC
Molecular Insight Research Foundation
Macula and Retina Institute
Los Angeles and Glendale, CA

Dosage should be individualized according to the disease under treatment and the general medical condition of each patient.

CF=counting fingers; FA=fluorescein angiography; HM=hand motions; ICG=indocyanine green chorioangiography; IVFA=intravenous fluorescein angiography; NSAID=non-steroidal anti-inflammatory drug; OD=right eye; OS=left eye; OU=both eyes; VA=visual acuity.

See full

INDICATION

Acthar® Gel (repository corticotropin injection) is indicated for severe acute and chronic allergic and inflammatory processes involving the eye and its adnexa such as: keratitis, iritis, iridocyclitis, diffuse posterior uveitis and choroiditis, optic neuritis, chorioretinitis, anterior segment inflammation.

Important Safety information

Contraindications

  • Acthar should never be administered intravenously
  • Administration of live or live attenuated vaccines is contraindicated in patients receiving immunosuppressive doses of Acthar
  • Acthar is contraindicated where congenital infections are suspected in infants

INDICATION

Acthar® Gel (repository corticotropin injection) is indicated for severe acute and chronic allergic and inflammatory processes involving the eye and its adnexa such as: keratitis, iritis, iridocyclitis, diffuse posterior uveitis and choroiditis, optic neuritis, chorioretinitis, anterior segment inflammation.

Important Safety information

Contraindications

  • Acthar should never be administered intravenously
  • Administration of live or live attenuated vaccines is contraindicated in patients receiving immunosuppressive doses of Acthar
  • Acthar is contraindicated where congenital infections are suspected in infants
  • Acthar is contraindicated in patients with scleroderma, osteoporosis, systemic fungal infections, ocular herpes simplex, recent surgery, history of or the presence of a peptic ulcer, congestive heart failure, uncontrolled hypertension, primary adrenocortical insufficiency, adrenocortical hyperfunction or sensitivity to proteins of porcine origins

Warnings and Precautions

  • The adverse effects of Acthar are related primarily to its steroidogenic effects
  • Acthar may increase susceptibility to new infection or reactivation of latent infections
  • Suppression of the hypothalamic-pituitary-axis (HPA) may occur following prolonged therapy with the potential for adrenal insufficiency after withdrawal of the medication. Adrenal insufficiency may be minimized by tapering of the dose when discontinuing treatment. During recovery of the adrenal gland patients should be protected from the stress (e.g. trauma or surgery) by the use of corticosteroids. Monitor patients for effects of HPA suppression after stopping treatment
  • Cushing’s syndrome may occur during therapy but generally resolves after therapy is stopped. Monitor patients for signs and symptoms
  • Acthar can cause elevation of blood pressure, salt and water retention, and hypokalemia. Blood pressure, sodium and potassium levels may need to be monitored
  • Acthar often acts by masking symptoms of other diseases/disorders. Monitor patients carefully during and for a period following discontinuation of therapy
  • Acthar can cause GI bleeding and gastric ulcer. There is also an increased risk for perforation in patients with certain gastrointestinal disorders. Monitor for signs of bleeding
  • Acthar may be associated with central nervous system effects ranging from euphoria, insomnia, irritability, mood swings, personality changes, and severe depression, and psychosis. Existing conditions may be aggravated
  • Patients with comorbid disease may have that disease worsened. Caution should be used when prescribing Acthar in patients with diabetes and myasthenia gravis
  • Prolonged use of Acthar may produce cataracts, glaucoma and secondary ocular infections. Monitor for signs and symptoms
  • Acthar is immunogenic and prolonged administration of Acthar may increase the risk of hypersensitivity reactions. Neutralizing antibodies with chronic administration may lead to loss of endogenous ACTH activity
  • There is an enhanced effect in patients with hypothyroidism and in those with cirrhosis of the liver
  • Long-term use may have negative effects on growth and physical development in children. Monitor pediatric patients
  • Decrease in bone density may occur. Bone density should be monitored for patients on long-term therapy
  • Pregnancy Class C: Acthar has been shown to have an embryocidal effect and should be used during pregnancy only if the potential benefit justifies the potential risk to the fetus

Adverse Reactions

  • Common adverse reactions for Acthar are similar to those of corticosteroids and include fluid retention, alteration in glucose tolerance, elevation in blood pressure, behavioral and mood changes, increased appetite and weight gain
  • Specific adverse reactions reported in IS clinical trials in infants and children under 2 years of age included: infection, hypertension, irritability, Cushingoid symptoms, constipation, diarrhea, vomiting, pyrexia, weight gain, increased appetite, decreased appetite, nasal congestion, acne, rash, and cardiac hypertrophy. Convulsions were also reported, but these may actually be occurring because some IS patients progress to other forms of seizures and IS sometimes mask other seizures, which become visible once the clinical spasms from IS resolve

Other adverse events reported are included in the full Prescribing Information.

Please see full Prescribing Information.