Refractory Depressive Disorder as the First Manifestation of Adolescent Sjögren's Syndrome, Successfully Treated with Rituximab.

Background: The psychiatric manifestations of Sjögren's syndrome are often overlooked despite their prevalence. They can be revelatory of the disease and include anxiety, depression, dementia and, rarely, psychosis. Case description: We report a case of 18-year-old female in whom a major depressive syndrome revealed primary Sjögren's disease, with a favourable outcome after treatment with rituximab. Conclusion: The diagnostic of Sjögren's syndrome should be considered in patients who present with unexplained and refractory neuropsychiatric symptoms, even in the absence of sicca symptoms. LEARNING POINTS: Psychiatric manifestations secondary to Sjögren's syndrome are not rare but often overlooked.They can be indicative of the disease and precede systemic signs by years.The diagnostic of Sjögren's syndrome should be considered in patients who present with unexplained and refractory neuropsychiatric symptoms, even in the absence of sicca symptoms.

Autoimmune Myelofibrosis Revealing a Systemic Lupus Erythematosus.

Background: Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease, characterised by multi-organ affections. Haematological involvement is a common manifestation of SLE, consisting of autoimmune peripheral cytopenia. Autoimmune myelofibrosis (AIMF) is a rare cause of cytopenia in SLE; it could precede or be concurrent with the diagnosis of SLE. There are few studies that describe this association. Case description: We report a case of AIMF revealing the diagnosis of SLE in 34-year-old female, presented with episodes of gingival bleeding associated with peripheral inflammatory polyarthralgia, photosensitivity and deterioration of general condition. Clinical examination revealed a soft pitting oedema in the lower limbs. Laboratory investigations showed a pancytopenia, inflammatory biological syndrome, with positive 24-hour proteinuria and anti-native DNA antibodies. A bone marrow biopsy showed diffuse myelofibrosis associated with maturation disorders and no tumour infiltrate. Renal biopsy revealed proliferative glomerulonephritis class III with immune deposits. Conclusion: The association of AIMF with SLE has been rarely reported, and it could be another cause for cytopenia in SLE. LEARNING POINTS: Autoimmune myelofibrosis can be associated with systemic lupus erythematosus (SLE), even though it is rare.This association should be considered when pancytopenia is not well controlled during SLE, prompting a bone marrow biopsy to confirm the diagnosis.The therapeutic management of this association is the same as that used in SLE.

Hypertrophic Pachymeningitis as an Unusual Cause of Headache and Sphincter Dysfunction in Systemic Lupus Erythematosus.

Hypertrophic pachymeningitis (HP) is an uncommon condition characterised by focal or diffuse thickening of the dura mater. An increasing number of cases have been reported of its association with underlying connective tissue diseases. It is a rare complication in systemic lupus erythematosus (SLE) and might be the initial and sole clinical manifestation. We report a case of a 21-year-old man presenting with febrile meningeal syndrome and sphincter dysfunction. Physical examination showed malar rash and joint pain. Biological assessment revealed a regenerative normocytic normochromic anaemia, a leucopenia and a lymphopenia. The 24-hour urine protein was positive at 0.6 g. Immunological evaluation revealed positive antinuclear, anti-Sm and anti-dsDNA antibodies. Brain and spinal magnetic resonance imaging showed hypertrophic pachymeningitis. Cerebrospinal fluid biochemistry was within normal limits. Renal biopsy revealed a mesangial proliferative lupus nephritis. The diagnosis of SLE with neurologic and renal involvement was established, and the patient was treated with intravenous methylprednisolone pulse, followed by oral prednisone in association with azathioprine and hydroxychloroquine. Considering the persistence of symptoms and MRI lesions after 6 months, a treatment with rituximab was initiated with good evolution. LEARNING POINTS: Hypertrophic pachymeningitis is a rare condition of diverse aetiologies.A workup including search for infectious, autoimmune and neoplastic aetiologies should be performed.It is an extremely rare complication in systemic lupus erythematosus and might be the initial and sole clinical manifestation.

Haemorrhagic Pericardial Effusion as the Presenting Symptom of Scurvy.

Introduction: Vitamin C deficiency (or scurvy) usually takes weeks to become apparent as cutaneous signs and impaired wound healing. Haemorrhagic pericarditis remains a rare complication of scurvy, which has never been reported as an isolated condition. We report the case of a haemorrhagic pericarditis revealing a vitamin C deficiency in a 56-year-old patient. Case description: A 56-year-old woman presented with a 2-week history of worsening chest pain and dyspnoea, with no significant medical history. Upon admission, the patient exhibited tachycardia, tachypnoea, low blood pressure, elevated jugular venous pressure, muffled heart sounds and multiple petechiae on her lower limbs. An ultrasound revealed a large pericardial effusion, and an emergency pericardiocentesis was performed, which yielded haemorrhagic fluid without atypical cells. An initial workup including haemoculture, PT and PTT, tuberculosis workup, autoantibodies, tumour markers and infectious disease was negative. A whole-body CT scan showed no evidence of tuberculosis or lymphoma. Additional testing showed that her vitamin C level was <3 umol/L. Following stabilisation, high-dose vitamin C therapy was initiated. Subsequently, she showed continued clinical improvement and remained asymptomatic upon her discharge. Discussion: While uncommon, it is crucial to investigate vitamin C deficiency when confronted with an unexplained haemorrhagic pericardial effusion, particularly in patients with risk factors. Conclusion: Our case highlights the significance of early detection of this condition in promptly addressing the diverse complications of scurvy, thereby enhancing the prognosis of a potentially fatal condition. LEARNING POINTS: Haemorrhagic pericarditis could be an initial indication of scurvy.Vitamin C deficiency must be included in the differential diagnostic of haemorrhagic tamponade, even in the absence of a typical signs and symptoms of scurvy.

[Stiff person syndrome associated with dermatitis herpetiformis: a case report]. / Syndrome de la personne raide associé à une dermatite herpétiforme: à propos d'un cas.

Stiff person syndrome (SPS) is a rare disease affecting the central nervous system which can be autoimmune, paraneoplastic or idiopathic in origin. Its typical classic presentation is characterized by progressive stiffness of the trunk and limbs, associated with spasms. The diagnosis is supported by the existence of continuous and spontaneous muscle activity on electroneuromyogram detection, the presence of serum anti-GAD antibodies, and a response to benzodiazepines. We report the case of a 46-year-old patient with a classic form of autoimmune stiff person syndrome associated with dermatitis herpetiformis.

[Drug-induced toxidermia in a patient receiving hydroxychloroquine as systemic therapy for primary Gougerot-Sjögren syndrome: a case report]. / Toxidermie médicamenteuse sous hydroxychloroquine pour traitement systémique d'un syndrome de Gougerot-Sjögren primaire: à propos d'un cas.

We here report the case of a 41-year-old female patient with maculopapular rash occurring a week after receiving hydroxychloroquine 400 mg for primary Gougerot-Sjögren syndrome with articular involvement. The patient had more than 1-year history of idiopathic minimal glomerular lesion, effectively treated with corticosteroid therapy. Maculopapular rashes resolved after hydroxychloroquine treatment was stopped and the patient was given hydrocortisone and desloratadine. Our case highlights the importance of prescribing low dose hydroxychloroquine in subjects with a history of kidney disease as well as of raising awareness and educating patients about side effects of hydroxychloroquine.

Myasthenia Gravis Revealing Hodgkin's Lymphoma.

INTRODUCTION: Myasthenia gravis is a rare autoimmune disease caused by autoantibodies directed against the synapses of the neuromuscular junction. PATIENT AND METHODS: We report the case of a young patient with myasthenia gravis associated with Hodgkin's lymphoma. RESULTS: A 22-year-old patient presented with a 2-month history of severe weakness associated with muscle fatigability and intermittent ptosis without dysphonia or respiratory signs. Clinical examination revealed generalized myasthenia. The EMG did not show post-synaptic block, and anti-acetylcholine receptor antibodies were elevated at 3 nmol/l (normal ≤0.3 nmol/l). CT of the thorax showed an anterior medial mass. Immunohistochemistry of the mass revealed mixed cellularity Hodgkin's lymphoma. CONCLUSION: The association of lymphomas with myasthenia gravis has been rarely reported. LEARNING POINTS: Myasthenia gravis is an autoimmune disease of the neuromuscular junction and can be associated with malignant haemopathy.The coexistence of myasthenia and Hodgkin's lymphoma is very rare.This association is a therapeutic challenge.