ObjectiveHypertrophic pachymeningitis (HP) is a relatively uncommon disease associated with focal or diffuse thickening of the dura mater secondary to underlying chronic inflammation.MethodsWe report ...a case of pachymeningitis revealing systemic lupus erythematosus (SLE) in a Tunisian male patient.ResultsA 46 year old patient presented with headaches and decreased visual acuity in the left eye. On further examination it was deemed to be secondary to underlying optic neuritis. Magnetic resonance imaging (MRI) revealed patchy pachymeningeal thickening and enhancement with areas of nodular thickening, pronounced around the left cavernous sinus.Our patient had a non specific small-joint arthritis and a papular rash of photosensitive distribution.A lumbar puncture was normal (one lymphocyte and no other cells, normal glucose (2.7mmol/l – serum level 4.2 mmol/l), normal protein (0.32 g/l), cryptococcal latex antigen test (CLAT) and India Ink negative, tuberculosis (TB) polymerase chain reaction negative and negative bacterial and TB culture). Further blood work-up revealed a lymphopaenia and an elevated erythrocyte sedimentation rate (90 mm in one hour) with normal complement 3, complement 4, treponema palladium antibody negative, ANCAs negative, normal IgG and IgG4 levels, normal serum calcium and other electrolytes. positive serology (antinuclear antibody positive (titre 1600 with speckled pattern), doublestranded DNA positive (100 IU/ml)), anti smith antibody was positive and a persistent lymphopaenia.A biopsy, done by neurosurgeons, have eliminated a cancer specillay lymphoma and granulomatosis. In light of the above findings, a diagnosis of SLE pachymeningitis was made using 2019 ACR EULAR Criteria.Our patient was treated with IVI methylprednisolone (750 mg daily for three days) and started on maintenance oral prednisone (60 mg), which was later weaned down, as well as a monthly dose of IVI cyclophosphamide (750 mg every month for six months). Our patient had an excellent, rapid response with complete resolution of headaches and neurology (excluding the optic atrophy) within one month. The patient showed good response to steroids and cyclophosphamide therapy. One should maintain a high index of suspicion to make the diagnosis in patients with SLE presenting with neurological dysfunction.ConclusionHP in patients with SLE is an extremely rare condition. At our knowledge, eight cases, three among them are Tunisians, were reported having HP in SLE. Steroids remain the backbone of treatment for HP in SLE with good success in isolated case reports. Other disease-modifying agents may be added based on response. It is imperative that the diagnosis is made early and treatment initiated to prevent further neurological sequelae.
La sarcoïdose est une granulomatose multi viscérale d'étiologie inconnue qui peut revêtir des tableaux cliniques et radiologiques diverses. Les localisations cérébrales bien que rares, peuvent se ...présenter sous forme pseudo-tumorale trompeuse. Nous rapportons l'observation d'un jeune adulte Tunisien hospitalisé pour hypertension intracrânienne en rapport avec une lésion pseudotumorale radiologique qui a révélé une sarcoïdose systémique.
Musculoskeletal involvement is the second common manifestation in Familial Mediterranean Fever patients (FMF). Sacroiliitis and spondyloarthritis (SpA) are a rare condition in this disease.
We ...present a 52-year-old woman with FMF and SpA and discuss particularities of this link. The patient had FMF since the age of 20 with intermittent inflammatory low back pain since the age of 28 and polyarthralgia of knees, left wrist and shoulders for a year. On examination there was limited extension of the left wrist and range of motion of the spine; pelvic examination and sacroiliac joints were normal. Molecular genetic analysis revealed a homozygous mutation of M649V. Laboratory investigations revealed an elevated C-reactive protein and erythrocyte sedimentation rate (31 mg/dl and 69 mm/1sth respectively). Further investigation showed a negative human leukocyte antigen-B27, antinuclear antibodies, anti-citrullinated peptides antibodies and rheumatoid factor. Pelvic and spine x-rays showed small areas of bilateral increased density and irregularity over the sacroiliac joints indicating grade 3 bilateral sacroiliitis and syndesmophytes. Magnetic resonance imaging of the spine revealed dorsal spondylitis and syndesmophytes. The diagnosis of SpA was retained according to Amor’s criteria. The patient was treated initially with non-steroidal anti-inflammatory drugs, physiotherapy and sulphasalazine which were ineffective after 3 months. The patient then received anti-tumor necrosis factor-α with good response.
Spndyloarthritis should be considered in FMF patients with associated sacroiliitis especially when there are leading symptoms and/or imaging abnormalities in the spine. Awareness of this co-existence is important among rheumatologsits for a timely and precise management plan.
Sarcoidosis is a multivisceral granulomatosis of unknown aetiology which may have various clinical and radiological manifestations. Cerebral sarcoidosis, although rare, can appears as a misleading ...pseudotumor. We report the case of a young Tunisian adult hospitalized for intracranial hypertension associated with pseudotumoral lesion on radiology, which was revealed to be systemic sarcoidosis.
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