Symptomatic Sarcoidosis

Pulmonary sarcoidosis and lupus pernio patient

Woman, aged 34 years, with sarcoidosis and lupus pernio¹

Image not of an actual patient.

Diagnosed with sarcoidosis after presenting with shortness of breath, cough, erythema nodosum on her legs, uveitis, and arthritis in legs and neck

Biopsies of leg lesions and sinuses demonstrated noncaseating granulomas
Chest X-ray revealed adenopathy
Spirometry within normal range

Presentation and Treatment History

Treatment With Acthar Gel

Safety

Presentation and treatment history¹

Treatment with prednisone

Leg nodules resolved with prednisone

Recurred with new nodules when prednisone was discontinued

Restarted prednisone at 10 mg/d; developed lupus pernio with painful nodules on cheeks, nose, and forearms

Prednisone increased to 40 mg/d plus methotrexate; skin lesions persisted

Discontinued methotrexate due to nausea and initiated infliximab

Diagnosed with infliximab-induced myositis and infliximab discontinued

Prednisone increased to 40 mg/d and initiated azathioprine 100 mg/d

Gradual improvement in skin lesions
Subsequently developed diffuse muscle achiness and weakness in her legs, arms, and back
- Symptoms progressed despite good control of her skin lesions
Blood testing demonstrated positive antinuclear antibody and anti-Jo-1 antibody presence
Markedly elevated levels were detected for serum creatine phosphokinase (CPK) and aldolase
HRCT scan showed no evidence of new or advancing interstitial lung disease
After 3 months, serum CPK decreased, but muscle aches and weakness persisted
Patient noted severe adverse effects from high-dose prednisone (weight gain, Cushingoid features, serious sleep disturbances, and mood changes)

Prednisone dose over time

Pulmonary sarcoidosis and lupus pernio patient prednisone dosage graph

Serum CPK levels over time

Pulmonary sarcoidosis and lupus pernio patient serum CPK levels graph

With the exception of prednisone, other therapies are not approved for the treatment of symptomatic sarcoidosis.²

Treatment with Acthar Gel¹*

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 2x/wk plus prednisone 15 mg/d and azathioprine 100 mg/d

Prednisone dose reduced to 10 mg/d^†

For 3 weeks, Acthar Gel was discontinued due to insurance issues

Acthar Gel was restarted

Prednisone dose reduced to 5 mg/d, followed by a 15-lb weight loss

After 6 months, skin lesions markedly improved with facial lesion resolution
- Musculoskeletal aches resolved and muscle strength increased significantly
- Serum CPK levels decreased and aldolase normalized
During discontinuation of Acthar Gel:
- Muscle aches and weakness recurred and serum CPK increased
- Skin nodules on legs worsened
After 2 months of restarting Acthar Gel, muscle symptoms resolved, skin lesions were near complete remission, and serum CPK decreased

Prednisone dose over time

Prednisone dosage in a pulmonary sarcoidosis and lupus pernio patient treated with Acthar Gel graph

Serum CPK levels over time

Serum CPK levels in a pulmonary sarcoidosis and lupus pernio patient treated with Acthar Gel graph

Safety Findings

No significant side effects were observed after the introduction of Acthar Gel.

^*Dosage and frequency should be individualized according to the medical condition, severity of the disease, and initial response of the patient.³

^†Prednisone tapering schedule is unknown.

Adapted from Zhou Y, Lower E, Li H, Baughman R —Resp Med Case Rep, 2015

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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.

Woman, aged 34 years, with sarcoidosis and lupus pernio1

Presentation and treatment history1

Treatment with prednisone

Prednisone dose over time

Serum CPK levels over time

Treatment with Acthar Gel1*

Prednisone dose over time

Serum CPK levels over time

Safety Findings

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IMPORTANT SAFETY INFORMATION

Contraindications

INDICATIONS

IMPORTANT SAFETY INFORMATION

Contraindications

INDICATIONS

Warnings and Precautions

Adverse Reactions

Woman, aged 34 years, with sarcoidosis and lupus pernio¹

Presentation and treatment history¹

Treatment with Acthar Gel¹*