In a recent published in the The Journal of the Association of Physicians of India, a team of physicians under Sanjay K Mahajan,from the Department of Medicine, IG Medical College, Shimla, Himachal Pradesh, have put forth a very rare case of Heerfordt's-Waldenström syndrome,a presentation of neurosarcoidosis characterized by parotid gland enlargement, facial palsy, anterior uveitis and fever.
Very rarely sarcoidosis can present with neurological complaints as presentation. Neurosarcoidosis (except 7th CN palsy) commonly leads to chronic disease and the patients, who require steroids initially, also have tendency to develop chronic disease.
There are only case reports of Heerfordt's-Waldenström Syndrome in the literature and randomised control trials of treatment are not available. The patients are treated as neurosarcoidosis.The patients of neurosarcoidosis should be treated with higher doses of steroids. Non-responders and/or those with intolerant to steroids, cytotoxic / immunosuppressive drugs can be used. Methotrexate, azathioprine, cyclosporine, mycophenolate mofetil, infliximab, all can be used. Methotrexate is one of most commonly used and studied drug of these all.
The 32 years old female presented with easy fatigability, facial asymmetry associated with worsening facial droop and slurring of speech. She also had dryness of mouth associated with difficulty in chewing food especially solid food and required to take a plenty of water for chewing and swallowing food for last 6 months. For last 4 months she had low grade fever, cough and dyspnoea on exertion. There was also history of diminution of vision, grittiness in eyes, redness of eyes, undocumented and unintentional weight loss and painless swelling bilateral side of the neck.
On examination, pallor, bilateral non tender enlarged parotids, multiple palpable firm non tender cervical lymph nodes and conjunctival congestion were noted. There were multiple hyper pigmented macules and papules over her shin bilaterally. In CNS examination, right sided facial infra nuclear palsy.
X-ray Chest revealed hilar LAP on right and Pulmonary function tests revealed mild restrictive pattern. HRCT chest revealed bilateral hilar, pre-tracheal and right para- tracheal lymphadenopathy , and ground glass haze in bilateral subpleural apices (Scadding stage III). Histopathology of salivary gland revealed multiple giant cells and naked non caseating granuloma .
On diagnosing the patient as having Heerfordt's-Waldenström Syndrome, oral steroids (Prednisolone 60 mg) were initiated. While tapering the dosage of steroid, she again had flare up of symptoms and dosage of Prednisolone again escalated. She improved, discharged from hospital on tapering dosages but developed intolerance to steroids and Methotrexate 10 mg once a week was added.
Finally , Her symptoms started improving and on follow up visits at 3 months and 6 months.
For full article follow the link: https://www.japi.org/x264e4c4/heerfordtwaldenstrm-syndrome
Primaryy source: The Journal of the Association of Physicians of India