Symptomatic Sarcoidosis

Progressive pulmonary sarcoidosis patient

Man, aged 55 years, with progressive pulmonary sarcoidosis^1,2

Image not of an actual patient.

Disease duration: 13 years

Presentation and Treatment History

Treatment With Acthar Gel

Safety

Presentation and treatment history^1,2

Treated with low-dose prednisone for several years; referred for worsening symptoms

Initially treated with prednisone and methotrexate

Started on adalimumab 40 mg/wk; symptoms improved

Withdrew prednisone and methotrexate

Insurance declined further coverage of adalimumab

Patient wanted to try prednisone monotherapy; reinitiated at 10 mg/d

Upon initiation of prednisone and methotrexate, there was continued worsening of pulmonary symptoms and chest imaging
After withdrawing from prednisone and methotrexate:
- Slowly worsening fibrotic changes on CT scan
- Reduced forced expiratory volume in the first second (FEV1)
After reinitiation of prednisone:
- Dyspnea progressively increased
- Worsening of pulmonary function and chest imaging
  - PET scan demonstrated activity in chest and abdominal nodes

With the exception of prednisone, other therapies are not approved for the treatment of symptomatic sarcoidosis.³

Treatment with Acthar Gel^1,2,*

These results are based on a single patient and may not be fully representative of outcomes in the overall patient population. This patient was on multiple therapies. The clinical outcomes may not be solely attributable to Acthar Gel.

Started on Acthar Gel 80 U twice weekly^†

Reduced dose to Acthar Gel 40 U twice weekly

After 2 months, reduced prednisone to 5 mg/d

Acthar Gel 40 U continued for 6 months

Patient had anxiety for 2 to 4 hours after injection with Acthar Gel 80 U twice weekly; anxiety improved with dose reduction, but still present for 30 minutes after injection
Prednisone dose reduced and Acthar Gel treatment continued for 6 months
- No significant change in chest X-ray and resolution of nodular infiltrates on high-resolution computed tomography (HRCT) scan
Reduced [¹⁸F]-2-fluoro-2-deoxy-D-glucose uptake on PET scan
Patient declined long-term treatment

Safety findings

Noted marked anxiety on the day of injection, resolved within 16 hours of injection; better with dose reduced from Acthar Gel 80 U twice weekly to 40 U twice weekly.

^*Dosage and frequency should be individualized according to the medical condition, severity of the disease, and initial response of the patient.⁴

^†lnformation on duration of 80 U of Acthar Gel is unknown.

Adapted from Baughman RP, Barney JB, O'Hare L, Lower EE —Respiratory Medicine, 2016

NEXT: Pulmonary Sarcoidosis and Lupus Pernio Case Study (Baughman et al 2016)

References

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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 origin

INDICATIONS

Acthar^® Gel (repository corticotropin injection) is indicated for:

Treatment during an exacerbation or as maintenance therapy in selected cases of systemic lupus erythematosus
Treatment during an exacerbation or as maintenance therapy in selected cases of dermatomyositis (polymyositis)
Adjunctive therapy for short-term administration (to tide the patient over an acute episode or exacerbation) in: psoriatic arthritis; rheumatoid arthritis, including juvenile rheumatoid arthritis (selected cases may require low-dose maintenance therapy); ankylosing spondylitis
Treatment of symptomatic sarcoidosis

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-adrenal (HPA) axis 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 to 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 masks 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 for additional Important Safety Information.

Man, aged 55 years, with progressive pulmonary sarcoidosis1,2

Presentation and treatment history1,2

Treatment with Acthar Gel1,2,*

Safety findings

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IMPORTANT SAFETY INFORMATION

Contraindications

INDICATIONS

IMPORTANT SAFETY INFORMATION

Contraindications

INDICATIONS

Warnings and Precautions

Adverse Reactions

Man, aged 55 years, with progressive pulmonary sarcoidosis^1,2

Presentation and treatment history^1,2

Treatment with Acthar Gel^1,2,*