A 47- year-old woman with no significant medical history was referred to our center with pain in the interphalangeal joints of the hands and the knees and ankles, erythematous nodules on shins, and swollen eyebrows. The symptoms had appeared 2 months before the patient's referral.
On clinical evaluation, polyarthritis along with symptoms of erythema nodosum-like nodules and low-grade fever was detected. Distinct red papules were visible above the eyebrows (Figure 1).
Results of routine hematological and biochemical tests including serum calcium were normal. Immunologic tests including anti-nuclear antibody (ANA), rheumatoid factor (RF), and tuberculin test were negative. Additionally, the erythrocyte sedimentation rate (ESR) was 51 mm/hr (normal range: 0-29 mm/hr for women), angiotensin-converting enzyme (ACE) level was 73 U/L (normal: less than 40 U/L), and C-reactive protein (CRP) level was 48 mg/L (normal: less than 10 mg/L). The patient reported a history of multiple tattooing over the eyebrows, and the last tattooing was performed 4 months before her present symptoms manifested.
Considering the presence of erythema nodosum and bilateral ankle arthritis, computed tomographic scan (CT) of thorax was performed, which showed bilateral hilar adenopathy with reticulonodular lesions in lower lobes of the lung (Figure 1). The laboratory test of tuberculosis performed via direct examination and culture of the sputum was negative. The diagnosis of Lofgren's syndrome, an acute form of sarcoidosis was confirmed based on the presence of the triad of erythema nodosum, bilateral hilar lymphadenopathy, and polyarthritis.
Considering a 99.95% positive predictive value of Lofgren's syndrome for the diagnosis of sarcoidosis, biopsy was not advised.
Prednisolone 30 mg/day along with azathioprine 100 mg/day as a steroid-sparing agent were administered to the patient. After a follow-up period of 6 weeks, improvement in joint and cutaneous symptoms was observed. However, a four-fold increase in the liver enzymes led to the discontinuation of azathioprine. Subsequently, the prednisolone dose was tapered to 2.5 mg weekly. However, at the next follow-up one month later, due to increased inflammation and erythema of the eyebrow lesion along with recurrence of previous symptoms, the dose of prednisolone was increased to 50 mg/day and intralesional corticosteroid injection was administered as well. Methotrexate (MTX), 15 mg injection per week, was also added to the treatment as an alternative steroid-sparing agent. However, no improvement was observed after 8 weeks.
Eventually, a biopsy of the eyebrow was recommended. However, due to the fear of a post-biopsy scar, the patient refused to undergo biopsy. Following the patient's complaint of increased appetite and weight gain but no improvement in symptoms, treatment with subcutaneous adalimumab 40 mg (one ampoule) every 2 weeks along with 10 mg prednisolone was started. The inflammation and erythematous papules disappeared in 2 weeks after the first injection (Figure 2). A follow-up chest CT after 4 weeks showed no significant abnormality. Subsequently, prednisolone was tapered and withdrawn over a period of 3 months, and adalimumab was continued as monotherapy.
Despite the early evidences of adalimumab effectiveness in sarcoidosis treatment, there is no standard protocol for such intervention. We administered two ampoules of adalimumab subcutaneously every month (80 mg in total), as suggested for rheumatoid arthritis.
After 2 months of adalimumab treatment, increasing intervals between the injections from 2 weeks to 3 weeks (one 40 mg adalimumab ampoule per 3 weeks) led to the re-emergence of symptoms, which required a subsequent interval reduction to 2 weeks again. After 9 months of follow-up, the patient is still receiving adalimumab as monotherapy, and no sign of disease recurrence has been observed.
Spinal CT scan of Thorax with IV contrast showing systemic hilar and mediastinal adenopathies with subtle reticulonodular lungs infiltration compatible with sarcoidosis.