Severe infections in patients with systemic lupus erythematosus from Tunisia: Prevalence and risk factors.

Since the improvement in management and the reduction of mortality caused by the disease activity, infections have represented the main cause of morbidity and mortality in systemic lupus erythematosus patients. We aimed to determine the prevalence and identify risk factors of severe infections in systemic lupus erythematosus patients. We conducted a retrospective study on 93 Tunisian lupus patients followed between 2010 and 2019. The mean age of the disease was 33.63 ± 13.76 years. Fifty-two patients had 118 infections and 16% of the infections were serious. The bivariate analysis revealed a positive correlation between the total cumulative doses of corticosteroids and the number of serious infections (p = 0.009). The multivariate study has shown that the number of flares (p = 0.03), pulmonary manifestations (p = 0.01), pleuritis (p = 0.001), and the total cumulative dose of corticosteroids (p = 0.04) were independent risk factors of severe infections (p < 0.001). The use of antimalarials exerted a protective effect from severe infection [OR = 0.19 (95% CI 0.03-0.9)] p < 0.03.

Neuro-meningeal cryptococcal infection revealing a multiple myeloma.

Rare cases of Cryptococcus have been documented in patients living with multiple myeloma. To date there has been no documented evidence of cryptococcosis revealing multiple myeloma. We reported a 63-year-old man who had a 2-months history continuous holocranial headaches, morning vomiting, complaining of blurred vision and fever. The biologic and the imaging showed a Cryptococcus meningoencephalitis. The search for a cause of immunodeficiency revealed a multiple myeloma. The diagnosis for Cryptococcus was confirmed according to an India ink stain, blood and cerebrospinal fluid culture. The patient's treatment for multiple myeloma was initiated with a chemotherapy regimen. The evolution was good without complication. Cryptococcosis, especially in the neuro-meningeal form, is a serious, deadly opportunistic infection. The search of an underlining immunodeficiency must be systematic. In this case, it was associated with early stage multiple myeloma.

Cardiac tamponade: an uncommon presenting feature of systemic lupus erythematosus (a case-based review).

Although pericarditis is the most prevalent cardiac involvement in systemic lupus erythematosus (SLE), cardiac tamponade is extremely infrequent notably as the first manifestation of the disease. Here we report the case of a 22-year-old woman presenting with cardiac tamponade as the initial presentation of SLE.

Clinical Features of Systemic Lupus Erythematosus in Tunisian Males.

PURPOSE: Systemic Lupus Erythematosus (SLE) in males is rare. Clinical and biological features, as well as, the outcome may differ comparatively to female patients. The purpose of our study is to define these clinical and biological features in Tunisian male patients presenting SLE. METHODS: A mono-centric, retrospective and descriptive study of 96 patients followed for SLE out of which 21 are males. A comparative study was then performed between male and female patients groups. RESULTS: Sex-ratio female/male was 3.6/1, the average age at diagnosis of SLE was 37.8±14 years. The most frequently noted clinical manifestations were: skin involvement (81%), renal involvement (71.4%) and joint damage (66.7). We observed a significant difference in clinical features between male and female patients (21 males and 76 females): renal failure (52% vs. 71.4%), serositis (23.8% vs. 2.7%), peripheral neuropathy (19% vs. 4%) and lung interstitial disease (14.3% vs. 1.3%). No significant difference was found in the positivity of serum antibodies between the two groups. Fifteen male patients (71.4%) had a SLEDAI score greater than or equal to 11, referring to high/very high disease activity. Out of the 32 patients who developed infectious complications during the course of the disease, 11 were male (52.4% of males). Concerning the male group, complete remission was observed in 10 patients (47.6%), while 10 others presented persistent sequella. We observed one death in the male group secondary to infective acute respiratory failure. CONCLUSION: SLE in male patients is rare and associated with poor prognosis. Disparity was observed in clinical and biological features as well as outcome in the different studies. In our study, we concluded that male lupus is more severe.

[Dehiscence of the lamina papyracea]. / Déhiscence de la lame papyracée.

We report the case of a 35 year old patient presenting with acute onset of right periocular pain with palpebral edema due to nose blowing with absence of trauma. Early assessment showed periorbital emphysema without ophthalmological involvement, normal pupillary light reflex and visual acuity. Computerized tomography (CT) scan of facial and orbital bones objectified right pneumo-orbita with orbital fat herniation via a partial agenesis of the lamina papyracea, without muscle incarceration. Emphysema gradually reduced after two weeks by applying pressure dressing associated with broad spectrum antibiotic therapy as well as following the rules for cleanliness (avoiding closed glottis efforts, nose blowing…). Pneumo-orbita is defined by the presence of orbital air. It is mainly caused by a trauma but few cases of spontaneous dehiscence have been reported. The opening of the lamina papyracea, acting as an antireflux valve, prevents air outlet. CT scan is essential to confirm the diagnosis. It most often has a spontaneous resolution but the presence of hyperpression, that can be responsible for ischemic optic neuropathy or occlusion of the central artery of the retina, require a rigorous monitoring in order to implement possible urgent decompressive intervention.

Non-coronary cardiac manifestations of systemic lupus erythematosus in adults: a comparative study.

Cardiac manifestations develop in the majority of patients with systemic lupus erythematosus (SLE) at some time during the course of their disease. This study was designed to assess cardiac abnormalities in patients with SLE by echocardiography and to compare the 2 groups of patients with and without cardiac manifestations. It was a transversal, descriptive study, conducted in the Internal Medicine Department at the Military Hospital of Tunis from January 2016 to June 2018. Eighty lupus patients, diagnosed on the basis of ACR (American college of rheumatology) criteria, were enrolled in the study and were evaluated by standard echocardiography with color Doppler. Out of 80 patients 42 (52%) had abnormal echocardiographic findings. Pericardial effusion was found in 55%, valvular abnormalities in 52% and 38% had pulmonary hypertension. Patients with pleural effusion (45 vs 15%) were more vulnerable to cardiac involvement as well as renal impairment (57 vs 44%). The difference, however, were not statistically significant (p>0.05) in the renal involvement. Active disease with low complement (80%) was associated with higher frequency of cardiac involvement than disease in remission (64%) but the result was not statistically significant (p=0.11). Cardiac abnormalities are very common in lupus patients even when clinically asymptomatic form. Echocardiography is an excellent non-invasive tool for cardiac evaluation. Their research must be systematic with echocardiography in order to reduce subsequent cardiac morbidity and mortality among the lupus patients.

[Dermatomyositis and Panniculitis: the function of immunoglobulins]. / Dermatomyosite et panniculite: place des immunoglobulines.

Panniculitis is an inflammatory disease of subcutaneous adipose tissue which is rarely associated with dermatomyositis. It can occur before, after or simultaneously with muscle damage. In most cases, the evolution of panniculitis and of other dermatomyositis affections is favorable with corticosteroids and/or immunosuppressants. We report the case of a 48 year-old patient who developed panniculitis lesions 2 months before having muscular signs. Skin involvement was resistant to corticosteroid treatment associated with immunosuppressants drugs. This led to the use of polyvalent immunoglobulin treatment improving both skin and muscle damage.

Sarcoidosis with heart involvement: a rare association of terrible prognosis, a report of two cases.

Sarcoidosis is a multisystemic disorder of unknown etiology which is characterized by the formation of non-caseating granulomas in involved tissues. Cardiac involvement is one of the least common manifestations and it can occur at any point of time during the course of sarcoidosis. Here we present the case of 2 patients with known sarcoidosis who develop cardiac abnormalities in the absence of known primary cardiac cause. In our report, we would like to draw attention to the importance of considering heart involvement in any case with systemic sarcoidosis especially in young age.

Coexistence of pyoderma gangrenosum and sweet's syndrome in a patient with ulcerative colitis.

Pyoderma gangrenosum (PG) and Sweet's Syndrome (SS) are inflammatory skin diseases caused by the accumulation of neutrophils in the skin and, rarely, in internal organs. These neutrophilic dermatosis (NDs) are distinguished by the existence of forms of transition or overlap. They are frequently associated to systemic diseases especially hematologic and gastrointestinal ones. We report a case of a patient with ulcerative colitis (UC) who successively developed two types of NDs: PG then SS. A 66 years old patient with a history of UC consulted in July 2012 for an erythematous swelling of the back of the right hand treated with antibiotics without improvement. At that time, bacteriological samples were negative. In October 2012, he was hospitalized for polyarthralgia and impaired general condition. In physical examination, he had vesiculobullous plaque of 10 cm long of the right hand and wrist, infiltrated erythematous plaque on the right leg and another topped with a large pustule at the left ankle. Skin biopsy showed at the back of the right hand an aspect of PG and at the infiltrated plaques of the ankle an aspect of SS. Prednisone was started with improvement of the skin lesions and a recovery condition. The combination of PG and SS has already been described in cases of hematologic malignancy and rarely in UC. There is also the notion of passage from a neutrophilic dermatosis to another. Indeed, a typical lesion initially of SS can evolve to a future PG. This case demonstrates that neutrophilic dermatoses form a continous spectrum of entities that may occur in UC.