Central neurological manifestations in a sample of Syrian patients with systemic lupus erythematosus: cross-sectional study.

Introduction: The authors aimed to study systemic lupus erythematosus (SLE) central neurological patterns and their correlations with the disease activity. Patients and methods: The authors' retrospective observational study was carried out on admitted SLE patients. The patients' demographic data, clinical examinations, laboratory tests, imaging studies, and systemic lupus erythematosus disease activity index (SLEDAI) were recorded. Results: Thirty-six SLE patients had neurological manifestations from 203 patients, but 8 patients were excluded. 90.2% were females. The age of neuro-lupus manifestation was 24.1+2.9 years. Neurological manifestations were the initial presentation in 25% of patients. General seizures were the frequent manifestation. SLEDAI was 29.51±18.43, while it was 18.3±9.2 among patients without neuropsychiatric systemic lupus erythematosus (NPSLE). Twenty-five percent of patients had pleocytosis on cerebrospinal fluid (CSF) analysis. Small lesions were seen in 57.1% of patients on brain MRIs, and large lesions were observed in 10.6%. These findings were compatible with the disease activity. Discussion: Central nervous system involvement ranged between 10 and 80%, and much more with active disease. The frequent finding was general seizures. Psychosis and cognitive impairment were relatively frequent. Adult NPSLE manifestations had developed before or around the time of SLE diagnosis and within the first year after diagnosis. These manifestations were directly correlated to the disease activity. Abnormality in CSF is characterized by slight pleocytosis, and elevation of protein with normal fructose. MRI is the neuroimaging test of choice for NPSLE in clinical practice. Conclusion: Central neurological involvement in SLE was seen early in the course of the disease, and correlating to the disease activity.

Primary Sjögren's syndrome without ocular manifestation: a case report.

Introduction and importance: Sjögren's Syndrome is a systemic immune disorder, manifested in dry eyes and mouth. Primary Sjögren's syndrome without ocular manifestation is seldom mentioned in the literature. Case presentation: The authors report a case of a 48-year-old female who complained of dryness of mouth and dysphagia for 6 months. Physical examinations showed dry lips with angular cheilitis, an erythematous tongue, and dry buccal mucosa, with multiple carious teeth. The salivary flow was scanty from the Stenson's and Wharton's ducts on both sides. Her ophthalmological examination was normal. Laboratory tests revealed leukopenia, anemia, thrombocytopenia, elevated levels of C-reactive protein and erythrocyte sedimentation rate, a strongly positive antinuclear antibody, anti-SS-A, anti-SS-, and rheumatic factor. Hyperechoic nodules in both parotids were shown by Ultrasonography. Salivary gland biopsy showed lymphocytic infiltration. Diagnosis of primary Sjögren's syndrome was made. She was treated with Pilocarpine 5 mg for 3 months, Vitamin C, and artificial saliva for oral dryness. She is under continuous follow-up with 50-60% relief, without any systemic complications. Discussion: Sjögren's Syndrome affects the exocrine glands causing dry mouth and eyes, and can cause systemic symptoms, including fatigue and joint pain. The incidence of ocular involvement among the reported cases is 86.1%, whereas our patient did not have any ocular involvement, and this represents a rare condition. The differential diagnosis included diabetes mellitus, hypothyroidism, chronic virology infection, and some medications that cause dryness, which were very much ruled out. Treatment of sicca symptoms involves artificial tears and medications that stimulate saliva flow while treatment of systemic disease includes corticosteroids, and various DMARDs, Rituximab. this disease has an increased relative risk for the development of B-cell non-Hodgkin's lymphoma. Therefore, patients need to be monitored, especially in the presence of risk factors. Conclusion: It is very important to diagnose this disorder early, using the various diagnostic criteria.

Treatment of recurrent uveitis and ankylosing spondylitis with Golimumab: a case report, Letter to Editor.

Introduction and importance: Uveitis, as an extra-articular presentation, is found in 23% of patients with ankylosing spondylitis (AS) and is a challenging disease to treat. Case presentation: The authors presented a 32-year-old male to the out-hospital, complaining of recurrent anterior uveitis 8 years earlier in his left eye, and suffered from inflammatory lumber pain for 2 years. So a diagnosis of AS after the failure of many therapeutic strategies, 50 mg /month subcutaneous Golimumab was started with clinical remission of AS and uveitis. Clinical discussion: The American College of Rheumatology recommends the use of etanercept and adalimumab in the treatment of recurrent uveitis in AS patients. Similarly, the European League Against Rheumatism recommended using Infliximab, Adalimumab, or Certolizumab to prevent the recurrence of uveitis recurrence. Till now, a case about treating refractory uveitis with Golimumab in AS patients was published. Conclusion: Golimumab was found to be effective in the treatment of uveitis associated with spondyloarthritis refractory at least one immunosuppressive drug.

Characterizing autoimmune uveitis to systemic diseases: a retrospective study from a Syrian tertiary reference center.

Background: Uveitis, a notable cause of severe visual impairment, is frequently characterized as infectious or noninfectious autoimmune uveitis (AU), the latter of which is commonly associated with younger individuals and systemic diseases. Despite the condition's widespread impact, there are substantial gaps in the comprehension of its pathogenesis, clinical presentation, and therapeutic response, particularly concerning systemic disease-associated uveitis. Aim of the study: The current study aims to bridge these gaps through an extensive examination of demographic and clinical features in AU patients, thereby informing future research, and therapeutic strategies, and improving patient outcomes. Methods: This retrospective observational study analyzed 261 patients with systemic disease-associated uveitis from January 2018 to December 2022 in Damascus, Syria. With diagnoses made using the Standardization of Uveitis Nomenclature Working Group Criteria, the study evaluated tailored treatment efficacy at the 24-month post-treatment mark, alongside comprehensive ophthalmic examinations, laboratory evaluations, and radiographic assessments. Results: In our study, included 87 patients with Systemic Disease-Associated Autoimmune Uveitis (SDA-AU). Women represented 64.36% of this group, and the mean age at diagnosis was 39.8±17.9 years (range 7-71) for men and 43.8±15.4 years (range 11-69). The most reported symptom was a painful red eye (52.87%). The onset of symptoms was sudden for 32.18% of patients, while 67.81% reported gradual development. Complications occurred in 33.33% of patients, including cataracts (41.37% of those with complications) and glaucoma (17.24%). Laboratory evaluations showed elevated inflammation markers in 66.66% of patients. Upon the 24-month assessment, 48.27% of patients achieved complete remission, 37.93% showed significant improvement, while disease worsened in 13.79% of cases. Conclusion: Our findings demonstrated that the presentation of AU in this cohort frequently precedes the diagnosis of systemic diseases, affirming the vital role of an early and accurate diagnosis of uveitis for the detection of underlying systemic conditions. In conclusion, our study underlines the significance of a comprehensive and multidisciplinary approach in the management of SD-AU, leading to improved prognosis and quality of life for patients.

A case of multiple myeloma misdiagnosed as seronegative rheumatoid arthritis: a rare case report.

Introduction: Multiple myeloma (MM) is a malignant plasma cell proliferation producing large numbers of monoclonal immunoglobulins. Typical MM symptoms include anemia, bone pain, hypercalcemia, and renal failure. Atypical presentations like joint involvement were rarely reported in the literature and may cause significant delays in treatment and adverse outcomes. Case presentation: The authors report a case of a 54-year-old female who presented with symmetrical polyarthritis and was misdiagnosed with rheumatoid arthritis. The diagnosis of MM was made after failing many treatments of rheumatoid arthritis and with further laboratory tests and procedures. Conclusion: This rare manifestation of MM carries a diagnostic challenge and causes a significant delay in treating such patients. Here, the authors report this unusual initial presentation with a review of several cases in the literature describing similar presentations.

Pancytopenia as a first presentation of late-onset systemic lupus erythematosus: a case report.

Introduction: Systemic lupus erythematosus (SLE) is a systemic immune disease that classically occurs in young to middle-aged women and may present with cutaneous, renal, haematologic, neurological, and/or other symptoms at the time of diagnosis. Late-onset SLE or SLE in the elderly is a subtype that differs from classic SLE in terms of age group, clinical symptoms, organ involvement and severity. Case presentation: A 63-year-old female noted to have pancytopenia. The patient was diagnosed with lupus upon obtaining clinical presentations and serological marker, along with high titres of the antinuclear antibody and/or anti-double-stranded DNA antibody. The patient was managed with glucocorticoids and mycophenolate mofetil therapy, which led to a rapid response. Discussion: Late-onset SLE accounts for 2-12% of SLE patients with a minimum age of onset of 50 years and older, leading to significant delays in diagnosis. Late-onset SLE differs from early-onset SLE in terms of sex and ethnicity prevalence, clinical symptoms and signs, development of organ damage, disease activity and severity, and prognosis. Some studies have also shown that late-stage SLE patients have higher rates of RF and anti-Ro/anti-La antibody positivity, lower complement titre, and higher incidence of elevated creatinine and decreased creatinine clearance. First-line treatment of pancytopenia is glucocorticoid. In refractory cases, rituximab and immunosuppressants can be used. Conclusion: It is important to assess any unusual presentation of SLEs when clinical suspicion remains high and conducting further laboratory and imaging investigation.

Prevalence, types, and characteristics of headache in Behcets disease without involvement of the central nervous system in the Syrian population: a case-cohort study.

Introduction: Oral ulcers, genital ulcers, and uveitis represent the typical trial of Behcet's disease (BD). It is well common on the Old Silk Road. The mucocutaneous lesions are the hallmark of BD, but neurological involvement is one of the severe symptoms. Headaches may be an early sign of BD neurological involvement. This study aims to investigate the headache prevalence and its types, and characteristics in a Syrian sample of BD patients. Methods: BD patients were clinically interviewed and examined to collect their information, symptoms, and signs. the International Study Group for Behcet's Disease diagnosis criteria was used to confirm the BD diagnosis. The International Classification of headaches was used when classifying the headaches. Results: One hundred twenty participants were included in the study. half of them were BD patients and the control group was also 60 participants. Among Syrian BD patients, 36.7% suffer from primary headaches and 36.7% suffer from secondary headaches. These findings were not significantly different between the BD patients and the healthy population. Our results showed that there was no statistically significant difference between the two groups. Conclusion: Headache should not be considered a predictor for neurological involvement among BD patients. Additional attention to BD patients or the specific treatment for headaches is not required and does not differ from the general population.

Nintedanib in systemic sclerosis treatment: a case report.

BACKGROUND: Nintedanib was approved for the treatment of scleroderma and scleroderma-related interstitial lung disease, as it decrease the forced expiratory volume. CASE PRESENTATION: A 48-year-old Asian female patient with systemic scleroderma 6 years ago developed breathlessness, nausea, heart palpation, and sudden severe occipital headache over the preceding week. She was receiving aspirin 81 mg/day and amlodipine 5 mg/day. Her diagnosis was diffuse scleroderma with pulmonary hypertension, interstitial lung involvement, and renal crisis. The modified Rodnan score was 18. We begin captopril at a dose of 12.5 mg, progressively escalating to 200 mg/day, and oral nintedanib was started at 150 mg. A total of 12 months after initiation of treatment, the patient's kidney function was normal. The pulmonary function tests improved. The modified Rodnan score was reduced to 10. We did not encounter any side effects in our case due to nintedanib treatment. CONCLUSION: Treatment with nintedanib is crucial for slowing lung function decline. Diarrhea was the most common adverse event. Scleroderma renal crisis occurs in 10% of patients and typically presents with an abrupt onset of hypertension and kidney failure. The optimal antihypertensive agent for scleroderma renal crisis is an ACE inhibitor. The mainstay of therapy in scleroderma renal crisis has been shown to improve or stabilize renal function in approximately 70% of patients and improve survival in nearly 80% at 1 year. Nintedanib may be effective, and fairly safe to use. Further exploration is anticipated to advance a new period of systemic sclerosis treatment.

Successful pregnancy after cyclophosphamide therapy for systemic lupus erythematosus: a case report.

Introduction and importance: The use of cyclophosphamide in women of childbearing age with severe systemic lupus erythematosus is normally indicated. However, cyclophosphamide is generally avoided during pregnancy due to the risk of teratogenicity, especially since its effect on fetal survival is poorly understood. This is a case report of a lupus patient exposed to cyclophosphamide during pregnancy. Case presentation: A 35-year-old woman with a history of lupus presented to our outpatient clinic in the 12th week of pregnancy for her sixth routine cyclophosphamide bolus. The fetal echocardiogram result with the gynecology consultation was normal with the recommendation for a medical termination of pregnancy, which has been refused by the patient. Shared decision-making with the patient included a discussion of the maternal risks of continuation of pregnancy in the setting of worsening systemic function and the fetal risks of definitive treatment with cyclophosphamide for a lupus flare and the patient decided to proceed with the pregnancy. Treatment with immunosuppressants, including azathioprine was initiated replacing cyclophosphamide with close monitoring of her and the fetus every month. Clinical discussion: The first trimester of pregnancy seems to be particularly susceptible to fetal malformations, although CPA effects on fetuses in later stages of pregnancy are also reported occasionally. Nonetheless, its repercussions on fetal survival remain poorly comprehended. Conclusion: In conclusion, exposing pregnancy to cyclophosphamide could end with pregnancy loss. Based on our experience, the survival of the fetus is strongly in doubt when cyclophosphamide is required to treat lupus in the mother. However, in rare cases, it could be without complications.

Systemic lupus erythematosus and Down syndrome: a case report and literature review.

Introduction: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that can involve any organ system, and may lead to significant morbidity and even mortality. Down syndrome (DS) is the most frequent genetic cause of intellectual disabilities, typically caused by the presence of an extra chromosome 21. Case presentation: A 47-year-old Syrian female of DS who complained of low-grade fever, oral aphthae, fatigue, and arthralgia three months before presentation. Although the patient was diagnosed with phenotypically and gynogenically DS, a milder mosaic type was identified. She appeared fatigued with a blood pressure 110/70 mmHg, pulse 104/min, temp 100 F, having oral ulcers, tenderness of joints on palpation, haemoglobin 9.4 g/dl, white blood cells 10.9/mm3, platelets 87 000×109/cm with C-reactive protein of 2,3 mg/dl, and an erythrocyte sedimentation rate of 68. Urea 33 mg/dl with creatinine 0.9. The rest of the tests were unremarkable. Urine analysis was normal. Discussion: The prevalence of SLE in DS in the literature was found only in five cases, with different presentations, in the last 36 years. In our case the patient presented with mild lupus manifestations and responded well to steroids and hydroxychloroquine. Also, In our case, an onset of SLE in an old DS (DS female patient), whereas SLE is more frequent in childbirth-aged women, in addition to that DS patients had a short life expectancy. Conclusions: DS is associated with a predisposition to developing connective tissue disorders, especially in young females. unfortunately, patients were not diagnosed in all five cases until later with a flare because of the cognitive defect.

Mixed connective tissue and ovarian cancer: a case report.

Background: Mixed connective tissue disease (MCTD) is characterized by high titres of distinct antibodies: U1 ribonucleoprotein with variable clinical features seen in rheumatoid arthritis, systemic lupus erythematosus, scleroderma, polymyositis, and dermatomyositis. Limited case reports revealed the association between MCTD and cancer, like lymphoma, lung cancers, and others. Case presentation: A 22-year-old female presented with enlargement of the abdomen and oedema of the lower extremities, gradually started 25 days The patient had been diagnosed to have rheumatoid arthritis. She was treated with 7.5 mg/week MTX for 6 months. Physical examination revealed: pallor, lower limb oedema, with synovitis and deformities of hands. The laboratory tests showed anaemia, elevated levels of creatine phosphokinase ESR, positivity of antinuclear antibody, anti-ds DNA, and antinuclear ribonucleoprotein. Urinary protein excretion was 1625 mg/24 h. Chest X-ray showed bilateral pleural effusion. Echocardiography revealed pericardial effusion Thoracic-abdominal and pelvic tomography showed a heterogeneous mass with a diameter of 5 × 6 cm at the expense of the right ovary. The mass was removed surgically, and a biopsy was taken, and was compatible with ovarian high-grade serous adenocarcinoma. A course of solumedrol 1 g/IV/3 days was applied, and then continue with 60 mg/day oral predlone. Later on discharge, she was taken 25 mg/day predlone, and methotrexate 10 mg. Conclusions: Our case showed that the patient had no risk factors for developing ovary cancer. On the contrary, our patient was a young, non-smoker, without any previous treatment before the RA diagnosis was taken, and finally, she had 3 children with full-term pregnancy, and well health. This case highlights the importance of maintaining a high index of suspicion for malignancy in MCTD patients. However, further investigation on the role of the immune system in the development of ovarian cancer in women with autoimmune diseases including MCTD remains necessary.

The mean platelet volume (MPV) in patients with systemic lupus erythematosus (SLE) and its correlation with disease activity: a cross-sectional/case-control study.

Introduction: Systemic lupus erythematosus (SLE) is a systemic disease, with unknown etiology. The authors aimed in this study to determine the connection between mean platelet volume (MPV) and disease activity of SLE. Although it has been studied in other rheumatological conditions like rheumatoid arthritis, its role in adult patients with SLE needs to be defined, especially in Syria. Materials and methods: The authors have included in a cross-sectional study, 80 patients with SLE and 80 controls.The SLE group was divided into two groups based on their disease activity index: the active disease group and the non-active disease group.In all groups, MPV and erythrocyte sedimentation rate (ESR) were analyzed. Clinical findings and Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) were evaluated in all patients. Results: MPV was significantly lower in SLE patients compared to the control group (8.49±1.2 fl and 10.0±0.5 fl, respectively) (P=0.001). A decrease in MPV below the cut-off value (7.2 fl) increased the risk of active disease by an odds ratio of 9.79 (95% CI: 3.4-27.9) (P<0.001). Conclusion: MPV may be a disease activity indicator in patients with SLE. MPV is reduced in patients with active SLE and presents an inverse correlation with SLEDAI.

Recurrent uveitis in a patient with CREST syndrome: a case report.

Introduction: Systemic sclerosis is a connective tissue condition presented with clinical manifestations, including ocular involvement in a small percentage. A few cases of uveitis were described in the literature, especially in CREST syndrome. Case presentation: The authors reported a case of a 48-year-old male with a blurred vision in his left eye that diagnosed with recurrent uveitis in the setting of CREST syndrome, treated with prednisolone, methotrexate, and golimumab, with improvement. Discussion: Ocular manifestations of systemic sclerosis include palpebral alterations, keratoconjunctivitis, sicca syndrome, cataracts, pinguecula, and blepharitis. To our knowledge, this is the fifth case of uveitis in a setting of CREST syndrome in the literature, and the first one in sex involvement, as the previous cases were females, and in its treatment by golimumab, an anti-tumor necrosis factor inhibitor. Conclusion: Although this association is low, we believe that it should be taken into consideration when treating these situations to obtain better treatment results. Collaboration between rheumatologists and ophthalmologists is necessary in deciding on treatment.

Antineutrophil cytoplasmic antibody-associated vasculitis with systemic sclerosis: a fatal case report.

Introduction and importance: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a rare co-occurrence with systemic sclerosis, in around 2.5-9% of patients. The clinical manifestations and prognosis of vasculitis in systemic sclerosis depend on organ involvement. It presented with rapidly progressive acute renal failure without malignant hypertension, and with pitting hand and foot ulcers get along with purpuric vasculitis in some cases reports. Reports had found that survival in those with pulmonary-renal syndrome is poor. However, high-dose corticosteroids and cyclophosphamide increase the survival percent in those patients. Case presentation: An 81-year-old female was admitted for newly diagnosed acute renal failure and highly elevated C-reactive protein levels. She was diagnosed with systemic sclerosis 8 years previously, with a 3-year history of interstitial lung disease, and a 2-year history of pulmonary hypertension. Treatment included home oxygen on demand, prednisone 5 mg/day, and azathioprine 75 mg daily. On physical examination, she had sclerodactyly, both extremities ulcers, severe livedo reticularis, and hyperpigmented papules on her hand and feet. Laboratory findings included a markedly positive MPO (p-ANCA), and anti-Scl-70. She was treated with pulse methylprednisolone without any improvement. After a day, she developed anuria and became comatose. Then, she developed cardiac arrest, leading to death. Clinical discussion: The presence of ANCA in systemic sclerosis patients ranges from 2.5 to 9% of systemic sclerosis patients. It presented with rapidly progressive acute renal failure without malignant hypertension, and with pitting hand and foot ulcers. The treatment with high-dose corticosteroids and cyclophosphamide is benefit. Survival in those with pulmonary-renal syndrome is poor. Conclusion: The presence of ANCA-associated vasculitis is rarely reported with scleroderma. It occurs most commonly in women with limited or Calcinosis, Raynaud phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasia (CREST) variants of scleroderma, as well as those with overlap features. Severe manifestations including pulmonary-renal syndrome and death may occur.

Ocular manifestations in COVID-19 infections: a case series.

Introduction: The prevalence of ocular abnormalities of COVID-19 is different according to different reports. However, currently available evidence on the presence of this virus in ocular secretions and its association with conjunctivitis is not well established. Objective: To reveal the ocular features among COVID-19 patients and to describe them with the findings of clinical data, inflammatory markers, and respiratory support therapy. Methods: Ocular symptoms were evaluated and recorded in 494 COV19 patients through questionnaire-style interviews, and an ophthalmologic examination. Data including age, sex, disease severity, and nasopharyngeal swab results were collected. Laboratory test values were reviewed. Patients with COVID-19 infections were classified into severe cases and mild cases. Results: The prevalence of ocular features was (2.83%). The most common features were conjunctival hyperaemia, epiphora, and foreign body sensation with itching. Patients with ocular manifestations on CPAP support therapy had higher rates of itching, lower rates of foreign body sensation. No differences were found in the levels of inflammatory marker. Meanwhile, patients used respiratory-aid therapy revealed higher values of white blood cells, platelet counts, erythrocyte sedimentation rate, C-reactive protein, ferritin, and lactate dehydrogenase. Discussion: Ocular involvement in COVID-19 and possibility of disease transmission through ocular tissues and secretions, has been registered in some reports, with a prevalence of 2-32%. The external and internal ocular parts are involved. Conclusion: Ocular features are not infrequent in COVID-19 patients.

A case report of sarcoidosis overlapped with Sjogren's syndrome.

Introduction and importance: The diagnosis of sarcoidosis and Sjögren's syndrome (SS) in the same patient is a challenge since sarcoidosis is considered an exclusion criterion for SS. Case presentation: The authors described a 62-year-old woman, who had SS for 8 years and presented with dry mouth, dry eyes, dyspnoea, and erythema nodosum. High resolution computed tomography of the chest showed symmetrical pulmonary micronodules, interstitial changes, and enlarged mediastinal lymph nodes. Anti-nuclear antibodies and anti-SSA antibodies were positive. Schermer's test was also positive. A biopsy of lung nodules revealed non-caseous granuloma. Salivary gland biopsy showed focal lymphocyte infiltration. Diagnosis of sarcoidosis and SS were done according to the classification criteria in this patient. Clinical discussion: Although the diagnosis of Sjogren requires the exclusion of conditions that cause dry eyes and mouth, such as sarcoidosis, Few studies have reported the coexistence of sarcoidosis and SS such as this case report. Conclusion: This case extends our understanding of overlapped SS with sarcoidosis and provides a referential value for clinical diagnosis.

Intestinal perforation as a first presentation of granulomatosis with polyangiitis: unusual case report.

INTRODUCTION: Granulomatosis with polyangiitis (GPA) vasculitis typically involves upper and lower airways and kidneys. Gastrointestinal involvement is rare, clinically reported as esophageal involvement, gastrointestinal hemorrhage, intestinal perforation, colitis, and pancreatitis. CASE PRESENTATION: We present a 36 old man, with intestinal perforation, laterally diagnosed as granulomatosis with polyangiitis. DISCUSSION: Only a few cases of intestinal perforation have been reported in the medical literature. GI symptoms may be present after the disease diagnosis in years. Intestinal perforation usually required surgery. The frequent kidney involvement of GPA is rapidly progressive glomerulonephritis, presented as acute kidney injury, usually accompanied by GI symptoms. Cyclophosphamide plus corticosteroids remain the effective therapy. The patient with GPA had a normal life expectancy due to the advances in treatment. Renal involvement and GI manifestations are considered bad prognosis predictors. CONCLUSION: This case report illustrates the need to consider intestinal perforation in patients with granulomatosis with polyangiitis, early surgical intervention and appropriate immunosuppressive therapy can be lifesaving.

Calciphylaxis during the course of psoriatic arthritis patient. Is it coincidence? A case report.

Introduction and importance: Calciphylaxis manifests clinically by skin ischemia and necrosis and histologically by calcification of dermal arterioles. Usually, it occurs in patients with end-stage renal disease on dialysis or in patients who had a kidney transplant. Here, the authors present a case of calciphylaxis occurring in a patient with psoriasis and psoriatic arthritis. Case presentation: A 66-year-old Syrian male with a history of psoriatic arthritis presented for evaluation of 2-month nonpainful ulcers on his feet and hands that were treated with warfarin. Biopsies confirmed the diagnosis of calciphylaxis. The patient received sodium thiosulfate, zoledronic acid, intralesional sodium thiosulfate injections, and an intravenous infusion of vitamin K with dramatic improvement. At the 3-month follow-up, his wounds had been completely remitted. Discussion: Nonuremic calciphylaxis occurs in many cases, like vitamin D administration, vitamin K antagonists' administration, chronic inflammation, and others. The association between calciphylaxis and psoriasis was reported only in four cases in the literature; meanwhile, this was the first case that described calciphylaxis in the setting of psoriatic arthritis. Conclusion: A suspicion of calciphylaxis should be maintained in patients with underlying inflammatory mechanism diseases.

Recovery fulminant hepatitis A in systemic juvenile idiopathic arthritis patient treated with tocilizumab: a case report.

Introduction: Systemic juvenile idiopathic arthritis (sJIA) is a rare systemic immune disorder that affects patients before 16 years of age. Several viruses have been reported to trigger this disease. Increased use of biologics, such as tocilizumab and anakinra, and decreased use of glucocorticoid may lead to improved outcomes in patients with sJIA. Serious liver injuries induced by tocilizumab include acute liver failure, hepatitis, and jaundice.Hepatitis A remains a highly prevalent disease in low-income countries. Case presentation: A 14-year-old Syrian child was diagnosed with sJIA and treated with different DMARDs, including MTX. Tocilizumab was then added as monotherapy and stopped after 12 doses after full diseases remission and normal laboratory tests. He presented with a very high alanine transferase, aspartate transferase, a spiked fever, and fatigue. He was infected with hepatitis A. Discussion: Liver abnormalities are uncommon in sJIA. Acute liver failure may develop a few months after the onset of sJIA.Although acute infections with the hepatitis A virus in children are self-limited, 0.1% of patients progress to fulminant hepatic failure, which spontaneously recovers in 40% of cases. No data are available concerning the coexistence of hepatitis A and sJIA. Our case was the first case presenting fulminant Hepatitis A in a sJIA patient treated with tocilizumab, which had recovered, and the authors initiated Anakinra as a treatment. Conclusion: Further follow-up and cohort studies are needed to find the exact prevalence and coexistence of Fulminant Hepatitis A in the coarse of sJIA treated with tocilizumab.

Male infertility with muscle weakness: a point of view.

Introduction and importance: The most common causes of infertility are idiopathic spermatogenetic disorders, occurring in multiple reproductive or systemic diseases. The underlying genetic disorders influence the treatment and transmission of the disease to the offspring. Case presentation: A 32-year-old Syrian male, married for 6 years, presented with primary infertility. The patient had a history of muscle dystrophy for 12 years. He had no previous medical or drug addiction or family history. He had gynecomastia. Semen analysis revealed oligospermia in the patient. Follicle-stimulating hormone was elevated. Gene analysis could not be done due to funding issues. The percutaneous testicular biopsy revealed hypospermatogenesis, atrophy, and marked hyalinization of the seminiferous tubules. Electromyography of the upper extremities demonstrated myotonic discharges, with a waxing-waning frequency, amplitude, and a characteristic 'engine revving' sound. Clinical discussion: Myotonic dystrophy (MD) is an autosomal dominant inheritance disease with adult onset. Muscle weakness is the predominant presenting feature, with early involvement of the distal limbs and neck muscles and a characteristic facial appearance.Systemic clinical manifestations may include cardiac conduction defects, cataracts, insulin resistance and diabetes, testicular atrophy with impaired spermatogenesis, and others. Testicular biopsy findings are specific. To our knowledge, this is the first case of male infertility associated with MD in Syria. However, there are no data on the prevalence of myotonic dystrophy type 1 (MD1) in Syria. Conclusion: The practicing physician should keep in mind the frequent association between MD and infertility.