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Alopecia is not a distinctive clinical sign in Wegener's granulomatosis and, as far as we know, to date no cases have been published describing this phenomenon.
We present the case of a 54 year old woman diagnosed with Wegener's granulomatosis, who in the first stage of her disease had alopecia and improved after treatment with cyclophosphamide and prednisone.
Nine months before her admission to our service, she had had paraesthesias, and leg pain and dysfunction. Electromyography showed some signs of sensorimotor polyneuropathy. She was given prednisone for 10 days (90 mg/day) and improved partially. Five months later, she started coughing up haemoptysic sputum, and had arthralgias in both hands, constitutional symptoms, and intense and diffuse hair loss (traction positive). Her temperature was 36.5°C, blood pressure 130/60 mm Hg, respirations 16, pulse 80 beats/min, and her weight was 44 kg.
Physical examination showed 2 cm abdomen hepatomegaly and leg distal muscular atrophy. 4/5 upper limb distal weakness, normal positional and vibratory sensitivity, paretic-spastic walk, and deep tendon reflexes increased diffusely with clonic reply. The erythrocyte sedimentation rate was 91 mm/1st h, platelets 678×109/l, C reactive protein 229 mg/l, rheumatoid factor 1 U/ml. A chest x ray examination showed a bilateral interstitial pattern with multiple fibre tracts of hilar origin and ulterior segment atelectasis. Chest computed tomography showed three small nodules located in the front segment of the right upper lobe and right middle lobe—one was 5 cm and the other two were 1 cm. We also noted scarring fibre tracts in the left upper lobe and lower lobe back basal regions. The antineutrophil cytoplasmic antibody cANCA titre was 1/160 U/ml (normal range 0–20), proteinase 3 was 133 U/l, and myeloperoxidase, antinuclear antibodies, SSA/Ro, SSB/La, RNP, and Scl-70 were negative. ECA was 23 U/l (normal range 8–55).
A diagnosis of Wegener's granulomatosis was made. The patient was treated with prednisone (60 mg/day) with normal tapering and 10 monthly cyclophosphamide pulses (each 500 mg/m2 each). The patient's symptoms, including respiratory, neurological involvement, and alopecia, improved. As Langford et al have described,1 after the last pulse of cyclophosphamide we added methotrexate (10 mg/week); the patient's condition deteriorated and she had mild atrophy on her right leg lateral side and slight alopecia, and the proteinase 3 level reached 100 U/l. We stopped methotrexate and gave oral cyclophosphamide (2 mg/kg/day); the neurological symptoms and alopecia then improved (traction negative) and proteinase 3 became normal.
Wegener's granulomatosis is one type of vasculitis whose mortality rate, if not treated, can be high (82%) with a survival rate of 5–12 months.2 Treatment of this disease with cyclophosphamide has increased the survival rate of patients,3 and the daily combination of high doses of prednisone and oral cyclophosphamide has proved to be very effective in more than 90% of cases, especially when used from its initial stage to its remission,.1 Because this combination has many side effects (in 42% of cases), other alternatives may need to be used, such as high doses of daily prednisone and monthly cyclophosphamide pulses, which have a higher degree of remission with fewer side effects, but also more relapses.4 The use of methotrexate for maintenance of remission is a successful alternative to oral cyclophosphamide with a lower percentage of relapses (16%),1 but in our patient that regimen was ineffective. Cyclophosphamide is an alkylating agent with cytotoxicity and immunosuppressive activity. Its main side effects are leucopenia, infections, vomiting5 and haemorrhagic cystitis.6 Alopecia is deemed to be one of the most common side effects of cyclophosphamide.5 The side effects are directly related to the doses given, so that these can be reduced with a weekly dose of a 500 mg pulse given for three months2; the length of exposure to the drug may be another factor to take into account.1 In our patient, alopecia appeared during the active stage of the disease. Once corticosteroids and cyclophosphamide were given, we were able to control the disease activity and cranial hair loss. We believe that the pilose follicle is another organ which nay be affected in Wegener's granulomatosis by a vasculitis of the scalp vessels; and although we did not perform a scalp biopsy, it seems likely that this disease might have caused the patient's hair loss.
The interesting aspect of this case is that the patient had Wegener's granulomatosis and alopecia and she improved with a treatment which included prednisone and cyclophosphamide.
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