Clinical features and outcomes in patients with human immunodeficiency virus-negative, Castleman disease: a single medical center study in Tunisia.

Introduction: Castleman disease (CD), known as angiofollicular lymph node hyperplasia, is an uncommon condition. The two most common histological subtypes are hyaline vascular and plasma cell. We performed a retrospective analysis to define the clinic-pathological features and survival of CD, which is quite rare focusing on the particularities of our series with a review of the recent literature. Methods: This is a retrospective study conducted in the department of internal medicine of Hedi Chaker hospital in Sfax, Tunisia over 25 years. The disease was histologically confirmed in all patients. For each file, we collected a set of data by filling in a pre-designed form. Results: 18 patients were included. There were 8 men and 10 women with a mean age of 42.8 years. CD was monocentric in 5 cases (28%) and multicentric in 13cases (72%). Clinically, peripheral adenopathy was present in 77.7% of patients and deep adenopathy in 72.2%. Systemic signs were found in 13patients, including general condition (4.4%), fever (16.6%), serositis (27.7%), and skin involvement (33.3%). A biological inflammatory syndrome accompanied the clinical picture in 66% of patients. Abnormalities in the blood count were found in 12cases (66%), with anemia in 11cases, thrombocytosis in 3cases, and hypereosinophilia in 3cases. Cutaneous Kaposi's sarcoma was associated with Castleman's disease in 2cases, Hodgkin's lymphoma, angioimmunoblastic T-cell lymphoma, and lymph node T-cell lymphoma were found in 1case respectively. 3 of the patients had associated connective tissue diseases such as Sjögren's syndrome in 2 cases and rheumatoid arthritis in 1case. HHV8 serology was positive in 1 case with a multicentric plasma cell form. Histologically, the plasma cell form represented 50% of cases, hyaline-vascular (39% of cases), and mixed (11% of cases). Therapeutically, high-dose corticosteroid therapy was initiated in 13 cases. As a second-line treatment, MOPP chemotherapy was used in 1case due to transformation into Hodgkin's lymphoma, and biotherapy (rituximab) was used in 2cases in the multicentric form. Surgical removal of superficial adenopathy was performed in 2patients with monocentric CD. Conclusion : Castleman's disease (CD) is a non-malignant lymphoproliferation of localized or multicentric form with a wide and heterogeneous clinical spectrum. Diagnosis can be difficult due to the lack of clinical and radiological specificity. Management depends on the clinical form involving surgical and/or medical management.

Central retinal vein occlusion : an uncommon complication in sarcoidosis.

Sarcoidosis is a multi-system granulomatosis of unknown etiology, defined by the presence of epithelioid and gigantocellular granulomas, without caseous necrosis. Ocular sarcoidosis manifests mainly as bilateral granulomatous anterior uveitis. Occlusion of the central retinal vein in sarcoidosis is a rare manifestation, which is the particularity of our observation. We report the case of a patient presenting with unilateral central retinal vein occlusion associated with granulomatous anterior uveitis on the same side. Systemic manifestations and further investigations led to the diagnosis of sarcoidosis.

ICOS gene polymorphisms in systemic lupus erythematosus: A case-control study.

The inducible T-cell costimulator (ICOS) may play an important role in adaptive immunity by regulating the interaction between T cells and antigen-presenting cells. Disruption of this molecule can lead to autoimmune diseases, in particular systemic lupus erythematosus (SLE). In this study, we aimed to explore the possible association between ICOS gene polymorphisms and SLE as well as their influence on disease susceptibility and clinical outcomes. A further objective was to assess the potential impact of these polymorphisms on RNA expression. A case-control study, including 151 patients with SLE, and 291 unrelated healthy controls (HC) matched in gender, and geographical origin, was performed to genotype two polymorphisms located in the ICOS gene: rs11889031 (-693 G/A) and rs10932029 (IVS1 + 173 T/C); using the polymerase chain reaction (PCR)-restriction fragment length polymorphism method. The different genotypes were validated by direct sequencing. The expression level of ICOS mRNA was assessed by quantitative PCR in peripheral blood mononuclear cells of SLE patients and HC. The results were analysed using Shesis and spss.20. Our results revealed a significant association between ICOS gene rs11889031 > CC genotype and SLE disease (codominant genetic model 1, (C/C vs. C/T), p = .001, odds ratio [OR] = 2.18 IC [1.36-3.49]); codominant genetic model 2, (C/C vs. T/T) p = .007, OR = 15.29 IC [1.97-118.5]); dominant genetic model, (C/C vs. C/T + T/T) p = .0001, OR = 2.44 IC [1.53-3.9]). Besides, there was a marginal association between rs11889031 > TT genotype and T allele with a protective role from SLE (recessive genetic model, p = .016, OR = 0.08 IC [0.01-0.63] and p = 7.6904E - 05, OR = 0.43 IC = [0.28-0.66], respectively). Moreover, statistical analysis indicated that the rs11889031 > CC genotype was linked with clinical and serological manifestations of SLE, including blood pressure, and anti-SSA antibodies production in SLE patients. However, the ICOS gene rs10932029 polymorphism was not associated with susceptibility to SLE. On the other side, we did not note any effect of the two selected polymorphisms on the level of ICOS mRNA gene expression. The study showed a significant predisposing association of the ICOS rs11889031 > CC genotype with SLE, in contrast to a protective effect of rs11889031 > TT genotype in Tunisian patients. Our results suggest that ICOS rs11889031 may act as a risk factor for SLE and could be used as a genetic susceptibility biomarker.

Granulomatous gingival manifestation in Melkersson Rosenthal syndrome: a case report.

Melkersson-Rosenthal syndrome is a rare condition of unknown etiology. It is characterized by a classical triad of symptoms: relapsing facial and lip swelling, facial palsy and a fissured tongue. We report the case of a 29-year-old female patient who presented with the above-mentioned symptoms of Melkersson-Rosenthal syndrome. However, clinical examination revealed an exceptional manifestation, which is the gingival hyperplasia. The symptoms were partially managed with systemic steroids and surgical resection of gingival hyperplasia. The most significant finding to emerge from our case is that gingival enlargement can be identified as a rare clinical feature of the MRS disease, which is confirmed difficult to be managed.

Acquired hemophilia A in a patient with adult-onset Still's disease: Successful treatment with steroids.

Acquired hemophilia A (AHA) is a potentially life-threatening hemorrhagic disorder with many etiologies. We report the first case in the literature describing the association of AHA with adult-onset Still's disease (AOSD).

Subglottic Masses Revealing Rosai-Dorfman Disease.

Rosai-Dorfman disease (RDD) is an uncommon lymphoproliferative disorder; RDD with oropharyngeal involvement is extremely rare, especially in adults. A 65-year-old woman with a complaint of progressive dyspnoea since 2016 presented with laryngeal involvement of RDD. A laryngoscopy examination revealed two solid, polypoid masses in the subglottic region, and a laryngeal biopsy concluded chronic inflammation without signs of malignancy. A second biopsy of axillary lymph nodes was performed, supporting the diagnosis of histiocytosis. The patient was treated with corticosteroids and then lost to follow-up. In 2019, she suffered from dyspnoea and a hoarse voice. Laryngoscopy examination showed a polypoid lesion causing airway obstruction at 70% and thickening of the lateral wall of the cavum. Physical examination found left axillary and submandibular adenopathy, and computed tomography revealed thickening of the supraglottic larynx narrowing the laryngeal pathway. Lymphadenectomy with immunohistochemical analysis revealed typical protein positive S-100 histiocytes and emperipolesis. The patient was treated with high doses of corticosteroids for six weeks then these were progressively decreased. The outcome was favourable; the laryngeal lesion disappeared after two weeks of treatment. LEARNING POINTS: Rosai-Dorfman disease is a rare cause of lymphadenopathy in adults. Extranodal presentation of the disease is possible mainly in the head and the neck region.The diagnosis is based on histological examination with the presence of histiocytes, which are S-100 positive, CD68 positive, and CD1a negative immunohistochemistry.The outcome is usually good in asymptomatic forms of the disease with no critical organ involved. The surgical resection is appropriate to the localised symptomatic form of the disease while corticosteroids are indicated in disseminated RDD as a first-line therapy.Inspired by our case, rare localisation of Rosai-Dorfman disease (RDD), led to clinical and therapeutic issues. That is why a review of the literature must be undertaken, to share experiences.

Evaluation of X chromosome inactivation in endemic Tunisian pemphigus foliaceus.

BACKGROUND: Almost 5% of the world's population develops an autoimmune disease (AID), it is considered the fourth leading cause of disability for women, who represent 78% of cases. The sex ratio when it comes to the most prevalent AID varies from 9:1 in systemic lupus erythematosus (SLE) to 13:1 in endemic Tunisian pemphigus foliaceus (PF). METHODS: To test the potential involvement of skewed x-inactivation in the pathogenesis of Tunisian PF, we analyzed the methylation status of a highly polymorphic CAG repeat in the androgen receptor gene and evaluated the x chromosome inactivation (XCI) patterns in peripheral blood-leukocyte-derived DNA samples of female patients with PF (n = 98) compared to healthy control (HC) subjects (n = 150), as well as female patients with SLE (n = 98) were enrolled as a reference group. RESULTS: XCI status was informative for 50 of the 98 PF patients (51%) and 70 of the 150 HC women (47%). Extremely skewed XCI patterns were more frequent in PF and SLEwomen than HC, but the difference was statistically significant only in women with SLE. No statistical difference was observed in XCI patterns between PF and SLE patients. PF phenotype-XCI correlation analysis revealed that (i) skewed XCI patterns may be involved in the disease's subtype and (ii) it was more pronounced in the endemic group than the sporadic one. Furthermore, preferential XCI showed an increase in heterozygote genotypes of PF's susceptibility polymorphisms in immunity-related X genes (FOXP3, AR, and TLR7) in PF patients compared to HC. CONCLUSION: Our results suggest that skewed XCI could lead to hemizygosity of X-linked alleles that might unmask X-linked deleterious alleles.

Agranulocytosis in systemic lupus erythematosus: A case report.

Agranulocytosis is a rare acute condition characterized by severe a < gft (neutropenia in which the neutrophils count is less than 100/mm3. It can be classified into two categories, inherited, and acquired. Acquired agranulocytosis is not commonly caused by auto-immune diseases such as systemic lupus erythematosus (SLE). We report a case of a patient suffering from agranulocytosis related to SLE at disease onset, associated with other rare disease involvements.

Differential Expression of Anti-Inflammatory RNA Binding Proteins in Lupus Nephritis.

Lupus nephritis (LN) is a type of immunological complex glomerulonephritis characterized by chronic renal inflammation which is exacerbated by infiltrating leukocytes and fueled by a variety of pro-inflammatory cytokines. A profound understanding of the pathogenesis of LN is necessary to identify the optimal molecular targets. The role of RNA-binding proteins (RBPs) in post-transcriptional gene regulation in the immune system is being explored in greater depth to better understand how this regulation is implicated in inflammatory and autoimmune diseases. Tristetraprolin (TTP), Roquin-1/2, and Regnase-1 are 3 RBPs that play a critical role in the regulation of pro-inflammatory mediators by gating the degradation and/or translational silencing of target mRNAs. In this study, we proposed to focus on the differential expression of these RBPs in immune cells and renal biopsies from LN patients, as well as their regulatory impact on a specific target. Herein, we highlight a novel target of anti-inflammatory treatment by revealing the mechanisms underlying RBP expression and the interaction between RBPs and their target RNAs.

Cavitary lung opacity of unusual cause during Behçet disease.

Pulmonary artery aneurysm must be evoked in front of any hemoptysis in a patient with Behçet disease as it requires urgent immunosuppressive therapy and often surgery.

Th17 and Th1 cells in systemic lupus erythematosus with focus on lupus nephritis.

Systemic lupus erythematosus (SLE) is a complex autoimmune disease characterized by T cells imbalance. Indeed, a correlation between levels of Th17 cells and disease activity has been reported. Our work aimed to study the functional association of subpopulations of Th cells and SLE with (lupus nephritis, LN) or without (lupus erythematosus, LE) renal involvement in Tunisian patients through the detection of intracellular cytokines and surface marker expression. The IL23R and RORC mRNA expression levels were evaluated. The level of Th17 and Th1 cells was higher in LE and LN patients compared to healthy controls (HC) (p = 0.007 and p = 0.018, respectively), while Th1/17 cells were increased only in LN patients compared to HC (p = 0.011). However, no significant difference was described in the mRNA expression levels of RORC and IL-23R between SLE and HC. Our findings suggest that the Th1/Th17 differentiation mechanisms are altered in SLE and that this imbalance should have an important influence on the development and severity of the disease.

Role of innate immune receptors TLR4 and TLR2 polymorphisms in systemic lupus erythematosus susceptibility.

AIM: Through their recognition of various bacterial cell wall components, TLR2 and TLR4 participate in the innate response and modulate the activation of adaptive immunity. Therefore, the genetic background of these receptors might play a crucial role in autoimmune diseases such as systemic lupus erythematosus (SLE). In this study, we investigated the possible association between polymorphisms within TLR2 and TLR4 genes with SLE susceptibility. MATERIAL AND METHODS: A total of 100 SLE patients and 200 unrelated healthy controls of the Tunisian population were enrolled in the study.TLR4rs4986790, TLR4rs4986791, and TLR2rs5743708 genotyping were performed using a polymerase chain reaction-restriction fragment length polymorphism method. The number of guanine-thymine (GT) repeat microsatellite in the intron 2 of TLR2 gene was analyzed by sequencing. RESULTS: We reported a lack of allelic and genotypic association between SNPs of TLR4 and TLR2 genes and SLE pathogenesis. No correlation was found with any SLE features. However, SLE susceptibility was associated with the GT repeat microsatellite polymorphism in the human TLR2 gene. Further subclassification of alleles into three subclasses revealed a significant association between the long-sized repeats ((GT) >23) and SLE. CONCLUSION: Though the results showed the absence of genetic association of TLR4 and TLR2 SNPs with the risk of developing SLE, we have identified a protective association between the microsatellite polymorphism in intron 2 of the TLR2 gene and SLE. Functionally, these (GT)n repeats may confer modifying effects or susceptibility to certain inflammatory conditions.

Annexin A1 and its receptor gene polymorphisms in systemic lupus erythematosus in the Tunisian population.

BACKGROUND: An association between ANXA1, FPR1 and FPR2 gene polymorphisms and the patho-physiology of many human diseases was suggested by numerous studies. OBJECTIVE: Our study aimed to evaluate association between common polymorphisms in the 9q21.13 and 19q13.41 and susceptibility to systemic lupus erythematosus (SLE) in the Tunisian population. MATERIALS: We performed a case-control study on 107 Tunisian SLE patients and 122 healthy controls to explore 9 polymorphisms of the three studied genes: rs2811226 and rs3739959 (ANXA1), rs5030880, rs1042229, rs1461765570, rs17849971, rs867228 (FPR1), rs17694990 and rs11666254 (FPR2). RESULTS: Four polymorphisms were found to be linked with SLE susceptibility: rs3739959-ANXA1 > G and GG (p = 0.021, OR = 1.73 and p = 0.014, OR = 2.06 respectively), rs867228-FPR1 > TT (p = 0.014, OR = 4.59), rs11666254-FPR2 > GG (p = 0.019, OR = 8.34) and rs17694990-FPR2 > T (p = 0.05, OR = 1.506). In homogenous groups of SLE patients depending on clinical manifestations and serological results, previous associations were confirmed with a panoply of manifestations of lupus including lupus nephritis, malar rash, mouth ulceration and hypocomplementia. CONCLUSION: Our study showed an association between ANXA1 > rs3739959, FPR1 > rs867228, FPR2 > rs11666254, FPR2 > rs17694990 and SLE susceptibility. Our results also showed a strong association between the two ANXA1 studied SNPs and LN which allowed us to suggest these two SNPs as biomarkers of LN development in SLE. Further research is needed to understand by which mechanism the gene variants affect susceptibility to SLE. Key Points • Lupus erythematosus is an autoimmune disease in which a panoply of factors are implicated • Annexin A1 interaction with its receptors are suggested as a target in therapy of a panoply of human disease in particular cancers • The present results highlighted the implication of Annexin A1 and its receptors gene polymorphisms in the physiopathology of lupus, in particular in the involvement of renal and cutaneous lesions.

[Tumor-like amyloidosis of the parotid : A case report].

INTRODUCTION: Tumor-like amyloidosis or amyloïdoma is a nodular lesion related to abundant amyloid deposits that can clinically mimic a malignant tumor. Its etiologic diagnosis requires searching an underlying infectious disease, a connective tissue disorder or a lymphoma. Parotid amyloïdoma is exceptional, and only four cases have been reported in the literature from 1988 to 2021 (PubMed research). CASE REPORT: We reported the case of a 60-year-old, diabetic and hypertensive woman, presenting an isolated swelling of the right parotid region without facial paralysis or cervical lymphadenopathy. A right superficial parotidectomy with a frozen section examination was performed. Histologically, the swelling was related to abundant amyloid deposits without tumor. On immunohistochemistry, amyloidosis was type AA. The association with the Sjögren's syndrome was confirmed. CONCLUSION: The association of parotid amyloïdoma with Sjögren's syndrome is a rare condition. The histologic diagnosis may be difficult in this case. Therefore, it is necessary in the case of amyloïdoma to confirm the diagnosis and carry out an etiological investigation to search for an underlying pathology.

Retrospective study of elderly onset sarcoidosis in Tunisian patients.

BACKGROUND: Sarcoidosis is a systemic granulomatous disease of unknown etiology. It affects mostly young adults. In the elderly, the presentation of this disease is different, often posing positive diagnosis problems. OBJECTIVES: We intend to describe the various clinical features and the management of sarcoidosis in elderly patients (age ≥65 years) compared to the younger ones. METHODS: We performed a retrospective, descriptive and comparative study in the Department of Internal Medicine in the University Hospital Hedi Chaker, Sfax, Tunisia, between 1996 and 2016. RESULTS: From a series of 80 patients, we found sixteen patients (20%) with sarcoidosis diagnosed after the age of 65 years. A female preponderance (81,25%) was noted. Intrathoracic involvement concerned 13 patients (81,3%). Extrapulmonary signs were also frequent (93,8%). The main extrathoracic manifestations were ganglionar involvement (75%), an alteration of the general health (31,3%), hepatic involvement (31,3%), cutaneous involvement (25%) and ocular involvement (25%). Biological manifestations were hypercalcemia, hypercalciuria, lymphopenia and hypergammaglobulinemia noted in respectively 12,5%, 12,5%, 31,3% and 50% of the cases. Angiotensin-converting enzyme(ACE) level was elevated in 100% of the patients. Lymphadenopathy and cutaneous biopsies were important contributing factors to diagnosis (respectively: 100% and 75% were positive). Oral corticosteroid therapy was required in 50% of cases. Evolution was marked by pulmonary fibrosis in two cases. Satisfactory course of the disease was observed in the other patients. CONCLUSION: Young and elderly subjects had common characteristics of sarcoidosis, except for more coexisting chronic morbidities, no erythema nodosum and more frequent high levels of ACE in the elderly group.

Pure extra-thoracic sarcoidosis: about 24 cases.

Introduction. Pure extra-thoracic sarcoidosis is rare and poorly characterized in the literature. Herein we present features of pure extra-thoracic sarcoidosis, in comparison with sarcoidosis with intra-thoracic involvement, in a population from the south of Tunisia. Methods. Retrospective, descriptive and comparative study of the patients presenting systemic sarcoidosis and attending the Department of Internal Medicine at the Hedi Chaker University Hospital, Sfax, Tunisia, for 21 years, from January 1996 to December 2016. Results. Twenty-four patients presented pure extra-thoracic sarcoidosis (30% of the cases of systemic sarcoidosis). There was a female predominance (79%). The mean age was 50 years. The main features were polyadenopathies (10 patients: 41.7%), cutaneous involvement (10 patients: 41.6%), inflammatory polyarthralgia (8 cases: 33.3%), general symptoms (6 patients: 25%), uveitis (6 cases: 25%), cholestasis (3 cases) and kidney involvement (2 cases). Lymphopenia, hypercalcemia, and hypercalciuria were observed each one in 12.5% of the cases, and high angiotensin converting enzyme (ACE) level in 38.5% of the cases. The histological proof was required in all the patients. Statistically significant associations identified were mainly hepatic involvement with general symptoms (p=0.035), peripheral lymphadenopathies (p=0.035) and kidney damage (p=0.022), and cutaneous involvement with articular manifestations (p=0.032). Systemic corticosteroids were used in half of the cases, with a good outcome in 21 cases, and recurrence in two cases. One patient was lost to follow up. The comparison to the form with mediastino-pulmonary involvement showed less lymphadenopathies (p=0.001), less lymphopenia (p=0.006), and less frequent use of corticosteroids (p=0.044). Conclusion. Our series was characterized by the frequency of the pure extra-thoracic form and the diversity of the systemic manifestations.

RNA receptors, TLR3 and TLR7, are potentially associated with SLE clinical features.

The influence of intracellular Toll-like-receptors (TLR), recognized as nucleic acid sensors, in the immunopathogenesis of systemic lupus erythematosus (SLE) is increasingly explored. Yet, the results of both functional and genetic studies remain conflictual. We evaluated the association between TLR3 and TLR7 genes selected variants and SLE and investigated the possible relationship with clinical and serological parameters. Then, we studied the genetic expression of these receptors, and if the TLR7 gene evades X chromosome inactivation (XCI). Our study covers 106 cases and 200 controls, genotyped using a polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) technique. TLR3 and TLR7 expression level was assessed by qPCR carried, respectively, on renal tissues and PBMC, and methylation status was evaluated by methylation-specific PCR. Results were statistically analysed using Shesis software, χ2 , and Mann-Whitney test. Significant associations with SLE susceptibility were found for the TLR3 rs3775291, rs5743305 and rs3775294 polymorphisms. Further subgroup analysis, TLR3 rs3775291 and rs3775294 polymorphisms were significantly associated with lupus nephritis (LN) and even correlate with the presence of auto-antibodies binding RNA molecules. SLE and LN were more common in men with rs3853839-G variant within TLR7 gene versus those carrying the C allele. Moreover, the role of the G allele in the TLR7 expression up-regulation was confirmed. However, gene expression analysis showed no significant differences in TLR3 and TLR7 mRNA levels between LN patient biopsies and healthy tissues (p > .05). When comparing patients and controls, no statistical difference was observed in XCI pattern. Otherwise, notable associations were raised between TLR3 and TLR7 gene variants and clinical and serological lupus features pointing towards the role of genetic background in the physiopathogenesis of the disease.

Vitamin D status and CYP27B1-1260 promoter polymorphism in Tunisian patients with systemic lupus erythematosus.

AIM: An association between serum vitamin D (Vit D) levels and systemic lupus erythematosus (SLE) has been reported by several studies that suggested the involvement of genetically determined characteristics of enzymes of vitamin D metabolism. Our study aimed to evaluate the relationship between 25 hydroxyvitamin D (25[OH]D) level, the most representative metabolite of VitD status, and polymorphism of the cytochrome P450, CYP27B1 gene, which influence vitamin D metabolism, and serum levels, in SLE Tunisian patients. MATERIAL AND METHODS: A cross-sectional study has been conducted in SLE patients (supplemented and not supplemented patients), matched to healthy controls by age and gender. The 25[OH]D serum level was measured by chemiluminescence assay and CYP27B1-1260 genetic polymorphism was carried out using PCR-RFLP methods. Statistical analysis was made using Shesis and SPSS.20 Software. RESULTS: Controls and Vit D not supplemented patients' groups presented the highest percentage of hypovitaminosis D. A significant difference in the mean level of circulating 25[OH]D between Vit D supplemented SLE patients and controls was observed (23.91 ng/ml and 7.18 ng/ml, respectively p = 3.4 105 ). Our results showed a correlation of high 25[OH]D level with complement component 3 levels and prednisolone drug. Moreover, the analysis of CYP27B1-1260 polymorphism in SLE patients and controls revealed a nonsignificant allelic or genotypic association. CONCLUSION: Despite the sunny climate, the high prevalence of Vit D deficiency is common in Tunisia. This hypovitaminosis D feature may affect the Vit D levels in our SLE patients but a direct association with the disease or with the genetically determined features remains unclear. More studies are needed to establish thresholds and susceptibility genes according to the characteristics of each population.

An Unusual Association: Takayasu's Arteritis and Tubulointerstitial Nephritis and Uveitis Syndrome.

The coexistence of two immune-mediated diseases in the same patient is unusual. Takayasu's arteritis (TA), which is a chronic granulomatous vasculitis, was discovered in a 47-year-old woman followed for tubulointerstitial nephritis and uveitis syndrome (TINU syndrome). We present the first case of this association. LEARNING POINTS: Rare autoimmune diseases can coexist in the same patient.Physical examination is important so that coexisting diseases can be identified.Takayasu's arteritis was discovered as an incidental finding in a patient followed fortubulointerstitial nephritis and uveitis syndrome.

Adult Still's disease and squamous cell carcinoma in a 69-year-old woman.

Adult-onset Still's disease (AOSD) has been recognized as a cause of fevers of unknown origin. Malignancies are the most important differential diagnoses of AOSD which has been rarely reported in association with cancer. The present paper undertakes the study of a 69-year-old Tunisian woman with AOSD according to the diagnostic criteria of Yamaguchi. She was treated by prednisone, then associated with methotrexate. 18 months later, she developed a squamous cell carcinoma treated with chemotherapy and radiotherapy.