Symptomatic Sarcoidosis
Pulmonary sarcoidosis and lupus pernio patient

Woman, aged 34 years, with sarcoidosis and lupus pernio1

Image not of an actual patient.

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 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

 

Serum CPK levels over time

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

Treatment with Acthar Gel1*

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

 

Serum CPK levels over time

Safety Findings

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

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.

*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