Table 3 Corticosteroids and immunossuppresants drugs used in SS patients

Systemic corticosteroids

In one case the treatment was changed to dantrolene for lack of efficacy [33]. In a case that had been previously treated with carbamazepine and otilonium bromide, systemic corticosteroids were prescribed for alopecia. However, treatment was interrupted a month later due to the appearance of adverse effects [13]. In one case, corticosteroids showed improvement of short duration and the treatment was changed [37]. In another case, a single pulse of corticosteroid diminished the frequency of muscle spasms during two weeks, but therapy was changed to cyclophosphamide and subsequently to azathioprine [51].

Corticosteroids have been combined with other immunosuppressants in 7 cases. In 5 patients as initial treatment [5, 37, 60, 67] and in two patients after other treatments [50, 62]. Corticosteroids were combined with immunoglobulin therapy in two patients, [60, 67] with mycophenolate mofetil in one patient [37], with cyclosporin in two patients reported by Rudnicka [5], with methotrexate in one patient [62] and with tacrolimus in another patient [50]. Other immunosuppressants have been used in combination to low doses of corticosterois, such as methotrexate [63], azathioprine [59]. Corticosteroids have also been used in combination with other drugs such as baclofen [33], phenytoin [5, 33,34,35], dantrolene [6, 46], carbamazepine [2, 41] and botulinum toxin [59]. In one patient initially treated with corticosteroids in monotherapy, amitriptyline was added later [61].

Immunoglobulin therapy

In one case, immunoglobulin therapy was used alone as a first option without improvement [64]. In two cases, immunoglobulin therapy was used in combination with corticosteroids [60, 67]. In one case, it was used as a first option in combination with dantrolene, but treatment was changed to pulsed treatment of methylprednisolone together with dantrolene [47]. In another case, immunoglobulin therapy was used as a second option after short-term improvement with corticoids and mycophenolate mofetil, with no response [37]. In another patient they were also used as second option treatment together with diazepam [51]. Endo used them as a second option with effect for 2 months and then changing to corticoids [50]. Arita used it as second option treatment after dantrolene with satisfactory response [3]. The treatment with immunoglobulin therapy was changed in six cases [37, 47, 50, 51, 64, 69]

Cyclosporin

In one of the cases, both alopecia and spasms improved. In the other case, improvement of spasms was achieved, but it was less effective for alopecia [5]

Mycophenolate mofetil

The initial treatment in this patient was with corticosteroids and mycophenolate mofetil. Later phenytoin was added. Treatment was changed due to short-term improvement. The treatment was changed to immunoglobulin without response, and subsequently, to plasmapheresis (5 cycles) with improvement in pain and cramps. Later this same case was treated with a combination of phenytoin, mycophenolate and corticosteroids [37], thus it can be deduced that mycophenolate and corticosteroids adminstarion was maintained throughout the course of the treatment.

Azathioprine

It was used after treatment with both botulinum toxin and corticosteroids to help lower the dose of corticosteroids [59]. In another case it was used after multiple treatments [51]

Plasmapheresis

It was used after short-term improvement with both corticosteroids and mycophenolate mofetil as the first option, and failure of immunoglobulin therapy as a second option. After plasmapheresis (5 cycles) the patient improved in pain and cramps [37]

Methotrexate

In one case it was used together with prednisone [62], and in another case it was added to corticosteroids due to loss of effect [63]

Tacrolimus

It was used together with corticosteroids after trying other treatments [50]

Cyclophosphamide

It was used together with diazepam after trying other treatments, and was later changed to azathioprine [51]