Hypertrophic pachymeningitis in systemic sclerosis sine scleroderma

Abstract

Dear Editor, Hypertrophic pachymeningitis is a clinical disorder characterized by chronic refractory headache with or without neurologic manifestations, resulting from localized or diffuse thickening of the dura mater.1, 2 These meningeal changes can be found in various autoimmune, neoplastic and infective conditions.1, 3 Idiopathic hypertrophic pachymeningitis is the diagnosis of exclusion. Systemic sclerosis sine scleroderma is a condition characterized by vasculopathy, visceral manifestations and serologic findings similar to systemic sclerosis in absence of skin sclerosis. Here we describe a patient with systemic sclerosis sine scleroderma and hypertrophic pachymeningitis. To the best of our knowledge there has been no previous report of pachymeningitis in a patient with systemic sclerosis. A 45-years-old woman, a diagnosed case of systemic sclerosis sine scleroderma, presented to us with a 4-month history of severe, throbbing, holocranial headache associated with nausea. There was no other significant medical history. General physical and systemic examination was unremarkable except for basal crackles in both lungs. Hemogram and biochemical parameters were within normal limits. Antinuclear antibody titre was 1 : 320 homogenous pattern and anti-Scl-70 was positive. Tests for syphilis, hepatitis B surface antigen, anti-hepatitis C virus and human immunodeficiency virus were negative. The levels of serum angiotensin-convertase enzyme were normal. Urinalysis and 24-h urinary calcium was within normal limits. Cerebrospinal fluid (CSF) examination showed normal opening pressure, no cells, protein 18 mg/dL, blood sugar 70 mg/dL (random blood sugar 96 mg/dL). Tuberculosis polymerase chain reaction, India ink stain and malignant cytology on CSF were negative. Chest radiograph was normal. High-resolution computed tomography (HRCT) chest scan revealed features of early interstitial lung disease. Pulmonary function tests were suggestive of restrictive lung disease and echocardiography was normal with no evidence of pulmonary arterial hypertension. Gadolinium-enhanced magnetic resonance imaging (MRI) revealed diffuse pachymeningeal thickening with enhancement throughout cerebral and cerebellar hemispheres, suggestive of hypertrophic pachymeningitis (Fig. 1). Subsequently, bronchoscopy with transbronchial lung biopsy was performed. Bronchoscopic alveolar lavage revealed alveolar macrophages (82%), lymphocytes (10%) and polymorphs (8%) and lung biopsy had normal histology. The patient did not give consent for meningeal biopsy. She was started on 50 mg oral corticosteroids daily. She showed marked clinical improvement with complete resolution of headache. Follow-up gadolinium-enhanced MRI of the brain performed in September 2010 revealed marked regression in dural enhancement (Fig. 2). Hypertrophic pachymeningitis is a rare clinical disorder which commonly presents with chronic headache with or without neurological manifestations. Neuroimaging is essential in making correct diagnosis. Gadolinium-enhanced MRI is superior to contrast-enhanced computed tomography (CT) scan for diagnoses. Corticosteroid therapy has been effective in alleviating symptoms and in arrest of clinical progression. In refractory cases, immunomodulators such as azathioprine, methotrexate and cyclophosphamide can be used.3, 4 Connective tissue diseases such as Wegener's granulomatosis, rheumatoid arthritis, mixed connective tissue disease, undifferentiated connective tissue disease, sarcoidosis, Behçet's disease and Sjögren syndrome have been reported to be associated with hypertrophic cranial pachymeningitis.1, 5-8 To conclude, hypertrophic pachymeningitis is a rare association with connective tissue diseases and it should always be suspected when a patient presents with chronic refractory headache in which other etiologies have been ruled out. To the best of our knowledge this is the first case of pachymeningitis with scleroderma.