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.

Cytokine and chemokine profiles in the sera of COVID-19 patients with different stages of severity.

INTRODUCTION: COVID-19 is a viral infection that disturbs the host's immune system and causes an overproduction of cytokines leading to a cytokine storm. The present study aimed to evaluate the serum levels of 27 protein biomarkers to determine their association with COVID-19 disease severity. METHODS: The serum levels of 89 patients with different degrees of COVID-19 disease severity [asymptomatic (n = 14), moderate (n = 14), severe (n = 30), and critical (n = 31)] and 14 healthy individuals were tested for a panel of 27 cytokines and chemokines using Luminex assay (27 Bio­Plex Pro Human Cytokine, Bio-rad™). RESULTS: IL-12, IL-2 and IL-13, as well as IL-17 and GM-CSF were clearly undetectable in asymptomatic patients. IL-8 levels were higher in asymptomatic compared with other groups. Very high levels of IL-6, IL-10 and the chemokines MIP-1α, MCP-1 and IP10 were associated with disease progression, while IL-4 tends to decrease with disease severity. CONCLUSION: Our study provides more evidence that excessive cytokine synthesis is linked to the disease progression.

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.

Anti-MDA5 antibody-positive dermatomyositis with mild encephalopathy with reversible splenial lesion: a possible rare association?

Central nervous system (CNS) involvement in dermatomyositis (DM) is seldom observed. However, there are very rare case reports of CNS involvement with juvenile dermatomyositis. Encephalopathy in DM may occur for a number of reasons, such as cerebral vasculitis and hypoperfusion/hypertensive encephalopathy, but mostly as a consequence of immunosuppressant treatment. We report here for the first time the case of a patient with two rare diseases, namely anti-MDA5 antibody-positive dermatomyositis and mild encephalopathy with reversible splenial lesion (MERS).

[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.

Necrotizing Fasciitis of the Breast Underlying an Autoimmune Disease.

Necrotizing fasciitis is a serious soft tissue infection that causes necrosis of the subcutaneous tissues and the muscle fascia. It is associated with a high mortality rate of around 25%. Necrotizing fasciitis of the breast is a rare entity. It is a rapidly progressive life-threatening condition which can lead to sepsis with multiple organ failure. We describe a case of necrotizing fasciitis of the right breast in a 48-year-old patient diagnosed with systemic sclerosis-rheumatoid arthritis overlap syndrome. LEARNING POINTS: Necrotizing fasciitis of the breast is a very challenging diagnosis due to its rarity and similarity with simple breast infection.It is a life-threatening condition which can lead to systemic shock and multiple organ dysfunction syndrome and death.It may occur in patients with rheumatic diseases treated with corticosteroids and immunosuppressant drugs.

Differential diagnosis of COVID-19 in symptomatic patients at the University Hospital Center Mohammed VI, Marrakesh.

INTRODUCTION: coronavirus disease 2019 caused by severe acute respiratory syndrome coronavirus 2 was first reported in Wuhan, China. Clinical spectrum of this disease has nonspecific symptoms shared by many other frequent infectious diseases of the respiratory tract and other respiratory tract diseases. This study explains the importance of differential diagnosis between COVID-19 and other lung diseases. METHODS: we analyzed in this study, the demographic features, clinical presentations, laboratory data and radiologic findings of the COVID-19 patients in comparison to those with other respiratory infections or diseases. RESULTS: the mean age of all patients was 38.04 years; 35 patients were later confirmed to be positive for SARS-CoV-2 infection. The most common symptoms reported by both groups included nonproductive cough and myalgia. Two of the non-COVID-19 patients were having below 92% oxygen saturation and low systolic blood pressure. The patients shared relatively similar laboratory findings except 3% of the non-COVID-19 patients who had lympho-neutropenia and 22.6% had high levels of C-reactive protein. Pulmonary tuberculosis and autoimmune disease respiratory disorder were suspected in 2 of the non-COVID-19 patients respectively. CONCLUSION: we emphasize the importance of good screening protocols, rapid detection of SARS-CoV-2 and other most common respiratory pathogens, which may help for a better control of COVID-19 spread and avoid delayed care of other lung diseases.

Thromboprophylaxis use and concordance with guidelines among medical and surgical patients in Morocco.

INTRODUCTION: No data are available on thromboprophylaxis use in Morocco. Our aim was to characterize patients at risk of venous thromboembolism and assess the rate of appropriate thromboprophylaxis. MATERIALS AND METHODS: This was a national, observational, multicentre survey of venous thromboembolism risk and thromboprophylaxis use in hospitalized patients. Data were collected on a predefined date in three university hospitals in Morocco using a standardized pre-printed form. Thromboembolic risk was assessed according to the American College of Chest Physicians (ACCP) 2008 guidelines. Patients were classified as "thromboprophylaxis indicated" or "thromboprophylaxis not indicated". RESULTS: 784 patients were analysed: 307 (39.2%) medical and 477 (60.8%) surgical. 421 (53.7%) were female. Medical patients were older than surgical patients (57.6 ± 11.5 vs. 46.2 ± 16.9 years, p<0.0001) and were more likely to have risk factors for thromboembolism (50.5% vs. 45.7% of patients, p=NS). 57% of patients without contraindications or bleeding risk were at risk of thromboembolism according to ACCP guidelines and thromboprophylaxis was prescribed to 42.8% of these patients. In contrast, 7.4% of patients with no thromboembolic risk also received thromboprophylaxis (proportion agreement: 61.0%; Kappa=0.296). Over half (54.5%) of medical patients at risk of thromboembolism did not receive thromboprophylaxis whereas 6.3% of those with no risk did receive it (proportion agreement: 76.4%; Kappa=0.433). These figures were 57.9% and 9.2%, respectively, for surgical patients (proportion agreement: 52.7%; Kappa=0.191). Thromboprophylaxis was given to 19.2% of patients with contraindications or a bleeding risk. CONCLUSIONS: Educational initiatives are imperative to inform doctors about appropriate thromboprophylaxis.

[Lupus nephritis: frequency, histological forms and predictors of renal disease]. / La néphropathie lupique: fréquence, forms histologiques et facteurs prédictifs de l'atteinte rénale.

BACKGROUND: Lupus nephritis is a common and serious damage in systemic lupus erythematosus. The aim of our study is to define the frequency and to identify the predictors of lupus nephritis in patients with systemic lupus erythematosus. METHODS: multicenter retrospective study on all systemic lupus erythematosus patients followed between 2005 and 2012. We analyzed the epidemiological, clinical and biological presentation at admission and during follow-up. We have identified two groups of patients: group 1 involving systemic lupus erythematosus without renal disease, group 2 : patient with lupus nephritis. RESULTS: We collected 105 patients including 100 women, 41 (39%) with lupus nephritis occurred after a period of 30±10 months of development of lupus. 4 patients (3%) were initially presented with acute renal failure requiring hemodialysis. The pathological findings of renal biopsy is respectively: class IV, III and II in 19 patients (63.3%), 4 patients (13.3%), and 4 patients (13.3%). Our patients with lupus nephritis received various treatment regimens: 38 patients (92.7%) received corticosteroids and combined with cyclophosphamide in 33 (80.5%) cases. Only young age, anemia and antinuclear antibodies were correlated with the risk of occurrence of renal damage. Predictors of the severity of lupus nephritis were the short time of occurrence of renal and renal failure. CONCLUSION: Lupus nephritis in our series is characterized by a severity of the clinical and biological explained by the frequency of proliferative forms, hence the necessity for early and adequate management.

Sacral pain as the initial symptom in primary Hodgkin's lymphoma of bone.

Progressive painless enlargement of peripheral lymph nodes is the most common presentation of Hodgkin's lymphoma. The bone involvement is seen particularly in the later stages, but very rarely in early stages of the disease. Primary osseous Hodgkin lymphoma is exceedingly rare. Here, we report a case of Primary osseous Hodgkin lymphoma in a 35-year-old male presenting with osteolytic lesions of iliac and sacral bone without other symptoms. Histopathology examination revealed Hodgkin's disease. All other investigations (including computed tomography of the chest, abdomen and pelvis, bone marrow aspiration) were negative for occult disease. Polychemotherapy was started and the symptoms resolved. The case report highlights the importance of clinical suspicion of primary Hodgkin lymphoma of the bone, especially those involving the pelvis bones.

Periorbital edema as initial manifestation of chronic cutaneous lupus erythematosus.

Periorbital edema occurs frequently in dermatomyositis, but it has rarely been noted in systemic systemic lupus erythematosus. We describe a patient who developed bilateral periorbital edema and erythema as the sole manifestation of systemic lupus erythematosus.