Successful hydroxychloroquine treatment in a patient with systemic lupus erythematosus-associated nailfold capillary abnormalities.

A 46-year-old woman with malar rash and periungual erythema, but no relevant medical history, visited our hospital. She had alopecia for 3 years. She had a discoid rash on her back. Urinalysis revealed no proteinuria. Blood test results indicated lymphopenia and hypocomplementaemia. She tested positive for ANA at a titre of 1:2560 (speckled pattern) and anti-Sm antibody but negative for both anti-dsDNA and aCL antibodies. Nailfold video-capillaroscopy (NVC) revealed nailfold capillary (NFC) abnormalities (Fig. 1a). She was diagnosed with SLE on the basis of the 1997 ACR classification criteria. After diagnosis, HCQ administration at a dose of 400 mg/day was commenced. After 3 months of treatment, the dermal lesions related to SLE had resolved almost completely. NVC indicated improvement of the NFC abnormalities (Fig. 1b). After 14 months, the lymphopenia, hypocomplementaemia and NFC were normalized (Fig. 1c).

. 1

Nailfold capillary findings

Nailfold video-capillaroscopy (NVC; optipix capillaroscopy, produced by Optilia Instruments AB, Sollentuna, Sweden) was performed from the index finger to the little finger of both hands. L2: left index finger; L3: left middle finger; L4: left ring finger; L5: left little finger; R2: right index finger; R3: right middle finger; R4: right ring finger; R5: right little finger. The figures for nailfold capillary microscopic examination are shown with a magnification of ×200. (a) The NVC test was performed when SLE was diagnosed. NVC showed many enlarged capillaries, giant capillaries and haemorrhages. (b) The test was conducted 3 months after the commencement of HCQ treatment. Giant capillaries had disappeared, but some enlarged capillaries and haemorrhage remained. Ramification was noted. (c) These are the test results 14 months after the introduction of HCQ treatment. Enlarged capillaries had largely disappeared. Only mild haemorrhage was detected. Most capillaries had normalized.

A correlation between skin disease activity and NFC abnormality has been reported in patients with SLE [1]. HCQ monotherapy can be useful for the maintenance of dermal lesions of SLE [2]. However, its efficacy for NFC abnormalities in patients with SLE is unknown. This is the first report of improvement in NFC abnormalities with HCQ monotherapy for SLE.

Funding: This work was supported in part by Mitsubishi Foundation (S.M.), Takeda Science Foundation (S.M.) and Mochida Memorial Foundation for Medical and Pharmaceutical Research (S.M.).

Disclosure statement: The authors have declared no conflicts of interest.

Data availability statement

Data are available upon reasonable request by any qualified researchers who engage in rigorous, independent scientific research, and will be provided following review and approval of a research proposal and Statistical Analysis Plan (SAP) and execution of a Data Sharing Agreement (DSA). All data relevant to the study are included in the article.