Systemic Granulomatosis Research Articles

Atypical sarcoidosis: About a case

Chronic lung consolidation is often associated with various respiratory pathologies, ranging from chronic infections to interstitial diseases such as sarcoidosis, which is a benign systemic granulomatosis of unknown etiology. Interstitial parenchymal involvement is usual. The pseudo-alveolar form is atypical, often presenting acutely, difficult to diagnose, but tends to evolve rapidly favorably under corticosteroid therapy. We report here a case of pseudo-alveolar sarcoidosis in a 58-year-old patient whose radiological and clinical presentation is unique and perplexing. In this study, we emphasize the rarity of this pseudo-alveolar sarcoidosis.

Sarcoidosis and Emergency Hospitalization

IntroductionSarcoidosis is an idiopathic systemic granulomatosis whose evolution is self-limiting in the majority of cases. However, it can progress to organ damage that menaces the vital or functional prognosis of patients. Sarcoidosis itself, but also its comorbidities, can pose a threat to the patient, require rapid initiation of treatment and justify emergency hospitalization. Research questionWhat are the reasons and prognosis of sarcoidosis patients hospitalized in emergency ? MethodsThe objectives of our study were to describe the causes of admission, and to identify predictors of mortality in sarcoidosis patients hospitalized in emergency. This is a retrospective monocentric study. We included patients hospitalized after a stay in the emergency room or in intensive care unit (ICU) or requiring an unscheduled hospitalization after a telephone advice or a consultation, between 01/01/2017 and 07/07/2020. ResultsWe identified 154 sarcoidosis patients hospitalized in emergency, among which 14 (9%) required the ICU. There were 81 men, with a median age of 55.0 [44.0; 67.0] years. Sarcoidosis was inaugural in 20 (14%) patients. The primary reason for hospitalization was lower respiratory infections in 32 (21%) patients, followed by acute pulmonary exacerbation of sarcoidosis in 17 (11%), suspected cardiac sarcoidosis in 13 (8.4%), and neurosarcoidosis in 12 (7.7%). The median length of stay was 6 [3.00; 10.0] days. In-hospital mortality rate was 3.9%. The 2-year transplantation-free survival following hospitalization was 86,8% [95% CI 81,4- 92,5]. The factors associated with a worse transplantation-free survival were Charlson comorbidity index (HR=1.29 [95% CI=1.04-1.61]; p=0.021), pulmonary hypertension (HR=2.53 [95% CI=1.10-5.83]; p=0.029), and oxygen therapy during hospitalization (HR=4.18 [95%CI=1.55-11.29]; p=0.005). InterpretationThe overall mortality of sarcoidosis patients hospitalized in emergency is high. The presence of comorbidities and the severity of respiratory failure, as reflected by oxygen requirement, are important prognostic determinants.

What is the potential of ChatGPT for qualified patient information? : Attempt of a structured analysis on the basis of a survey regarding complementary and alternative medicine (CAM) in rheumatology

The chatbot ChatGPT represents amilestone in the interaction between humans and large databases that are accessible via the internet. It facilitates the answering of complex questions by enabling acommunication in everyday language. Therefore, it is apotential source of information for those who are affected by rheumatic diseases. The aim of our investigation was to find out whether ChatGPT (version3.5) is capable of giving qualified answers regarding the application of specific methods of complementary and alternative medicine (CAM) in three rheumatic diseases: rheumatoid arthritis (RA), systemic lupus erythematosus (SLE) and granulomatosis with polyangiitis (GPA). In addition, it was investigated how the answers of the chatbot were influenced by the wording of the question. The questioning of ChatGPT was performed in three parts. PartA consisted of an open question regarding the best way of treatment of the respective disease. In partB, the questions were directed towards possible indications for the application of CAM in general in one of the three disorders. In partC, the chatbot was asked for specific recommendations regarding one of three CAM methods: homeopathy, ayurvedic medicine and herbal medicine. Questions in partsB andC were expressed in two modifications: firstly, it was asked whether the specific CAM was applicable at all in certain rheumatic diseases. The second question asked which procedure of the respective CAM method worked best in the specific disease. The validity of the answers was checked by using the ChatGPT reliability score, aLikert scale ranging from1 (lowest validity) to7 (highest validity). The answers to the open questions of partA had the highest validity. In partsB andC, ChatGPT suggested avariety of CAM applications that lacked scientific evidence. The validity of the answers depended on the wording of the questions. If the question suggested the inclination to apply acertain CAM, the answers often lacked the information of missing evidence and were graded with lower score values. The answers of ChatGPT (version3.5) regarding the applicability of CAM in selected rheumatic diseases are not convincingly based on scientific evidence. In addition, the wording of the questions affects the validity of the information. Currently, an uncritical application of ChatGPT as an instrument for patient information cannot be recommended.

First Report on Chronic Granulomatous Disease from Nepal and a Review of CYBC1 Deficiency.

Chronic granulomatous disease (CGD) primarily results from inherited defects in components of the nicotinamide adenine dinucleotide phosphate oxidase enzyme complex. These include gene defects in cytochrome B-245/558 subunit α/β and neutrophil cytosolic factors 1, 2, and 4. Recently, homozygous loss-of-function variants in cytochrome B-245 chaperone 1 gene (CYBC1) have been discovered to cause CGD (CYBC1-CGD). Data on variant-proven CGD from low-income countries, the most underprivileged regions of the world, remain sparse due to numerous constraints. Herein, we report the first cohort of patients with CGD from Nepal, a low-income country in the Himalayas' challenging terrain. Our report includes a description of a new case of CYBC1 deficiency who was first diagnosed with CGD at our center. Only a dozen cases of CYBC1-CGD have been described in the literature thus far which have been reviewed comprehensively herein. Most of these patients have had significant infections and autoimmune/inflammatory manifestations. Pulmonary and invasive/disseminated bacterial/fungal infections were the most common followed by skin and soft-tissue infections. Inflammatory bowel disease (IBD) was the most common inflammatory manifestation (median age at diagnosis: 9 years) followed by episodes of recurrent/prolonged fever. Other autoimmune/inflammatory manifestations reported in CYBC1-CGD include acute pancreatitis, hemophagocytic lymphohistiocytosis, systemic granulomatosis, interstitial lung disease, arthritis, autoimmune hemolytic anemia, uveitis, nephritis, and eczema. Our analysis shows that patients with CYBC1-CGD are at a significantly higher risk of IBD-like illness as compared to other forms of CGD which merits further confirmatory studies in the future.

Anicteric Cholestasis Syndrome with Pruritus Revealing Systemic Sarcoidosis

Introduction: Sarcoidosis is a systemic granulomatosis of unknown aetiology characterised by the formation of non-caseating granulomas in organs. Hepatic involvement can be difficult to diagnose. Material and method: We report the case of a 30-year-old female patient with a long history of intractable pruritus revealing systemic sarcoidosis. Case report: A 30-year-old female patient presented with a persistent pruritus without jaundice or fever. Examination revealed tender hepatomegaly and scratch lesions. Laboratory tests revealed elevated liver enzymes with cholestasis, and serum protein electrophoresis showed an inflammatory profile. The different viral hepatitis tests were non-contributory. Alpha fetoprotein was normal. Abdominal ultrasound and CT scans revealed a heteronodular hepatomegaly. The first liver biopsy was consistent with autoimmune hepatitis lesions. The disease improved with general corticosteroid therapy and azathioprine. Two years later, with the persistence of the pruritus, blood calcium levels and angiotensin-converting enzyme tests were found to be elevated. A new liver biopsy showed epitheliogigantocellular granulomas without caseous necrosis. A thoracic CT scan revealed mediastinal adenopathy. This suggested systemic sarcoidosis. The patient progressed well clinically and radiologically on general corticosteroids with adjuvant therapy and azathioprine. Conclusion: Sarcoidosis is a systemic disease that can affect most tissues and organs. Hepatic involvement is frequent and more often difficult to diagnose.

A Rare Case of Retroperitoneal Sarcoidosis

Sarcoidosis is a systemic granulomatosis of undetermined etiology characterised by the formation of epithelioid and gigantocellular granulomas without caseous necrosis in the affected organs. Our case has a history of sarcoidosis with left flank pain. CT scan founded a left hydronephrosis secondary to a retroperitoneal mass encasing the lumbar ureter which was shunted by a double j stent. Biopsy of the mass showed fibrous tissue with epithelioid and gigantocellular granuloma without caseous necrosis. Our patient is an extremely rare case report.

LES ASPECTS EPIDEMIOLOGIQUES ET EVOLUTIFS DE LA SARCOIDOSE MEDIASTINOPULMONAIRE AU SEIN DU SERVICE DE PNEUMOLOGIE CHU HASSAN II FES

Introduction:Sarcoidosis is a non-necrotizing systemic granulomatosis of unknown cause characterized by a predominant pulmonary tropism. It is a disease of very variable manifestation and evolution. Its evolutionary course is difficult to predict: from spontaneous resolution in the absence of treatment to progressive organ destruction despite several lines of therapy. Objectives: The aim of our study is to describe the evolutionary profile of mediastinopulmonary sarcoidosis followed in the pneumology department of CHU Hassan II Fez. Material and methods:This is a retrospective study, covering the cases of mediastinopulmonary sarcoidosis followed in our department between January 2016 and December 2019 we required a follow-up period of at least 3 years to be able to retain the cure. Results:We collected 41 patients, the average age was 45.68 years (28-65), with a clear female predominance, 95 ,1% of women, type I was found in 12.2% of cases, type II in 73.2% of cases, type III in 7.3% of cases, and type IV in 7.3% of cases also. Histological confirmation was obtained by mediastinoscopy in 41.5% of cases, and by staged bronchial biopsies in 46.4% of cases. We opted for a therapeutic abstention in 46.3% of the cases (19 patients), 3 of them required treatment during their follow-ups. 22 patients required treatment with oral corticosteroids from the start, 4 relapses were noted after stopping corticosteroids, 4 patients required a second line of azathioprine, and 15 patients were cured after at least 3 years of no treatment.

Wegener’s Granulomatosis Revealed by Bilateral Orbital Inflammatory Pseudotumor: a Case Report and Literature Review

Purpose: To describe the chronology and the extent of orbital involvement in a case of granulomatosis with polyangiitis (GPA). Methods: Descriptive case report and literature review. Results: A thirty-two years old male consulted with the following complaints: A retro-orbital discomfort in both eyes, a bilateral fast progressive proptosis and a decrease in visual acuity in the left eye. Eye examination revealed a proptosis, ptosis, epiphora and a motility restriction and peripheral corneal ulcer infiltrates with nodular scleritis in the left eye. A palpable orbital mass was located in the sub temporal orbit in both eyes. Orbit magnetic resonance imaging (MRI) unveiled a diffuse and elongated enlargement of both lacrimal glands with enhancing mass in the superior lateral aspect of both eyes globe. The diagnosis of an orbital pseudotumor was suspected. The biological work-up revealed a high blood levels of inflammation markers and antineutrophil cytoplasmic antibodies (c-ANCA). The diagnostic of active systemic granulomatosis with polyangiitis was then made. Conclusion: As seen in this case, clinicians should consider a differential diagnosis of granulomatosis with polyangiitis in patients with an orbital pseudotumor mimic aspect.

A New Parasitic Archamoeba Causing Systemic Granulomatous Disease in Goldfish Extends the Diversity of Pathogenic Endolimax spp.

Endolimax is a genus of intestinal amoebae which stands among the least known human protists. Previous studies on amoebic systemic granulomatosis of a marine fish (Solea senegalensis) resulted in the unexpected characterization of a new organism which was related to Endolimax and named E. piscium. The existence of multiple reports of systemic granulomatosis caused presumptively by unidentified amoebae in goldfish lead us to investigate the organism involved in goldfish disease. Analysed goldfish presented small whitish nodules in the kidney, which correspond to chronic granulomatous inflammatory reactions with a ring-layer of amoebae in the periphery. Amoebae were amitochondriate and were located in a parasitophorous vacuole within macrophages, as previous studies on this condition in goldfish and other freshwater fish pointed out. SSU rDNA characterization confirmed a new Endolimax lineage which appears closely related to E. piscium, but the molecular evidence, distinct pathological features and lack of ecological overlapping between the hosts support their assignment to a new species, E. carassius. The results support the existence of a considerable unexplored diversity of Endolimax spp. among fish, and their proper characterization can contribute to an understanding of Archamoebae evolution and pathogenic potential.

Investigation of systemic granulomatosis in cultured meagre, Argyrosomus regius, using clinical metagenomics

Systemic granulomatosis is a peculiar disease of unknown etiology affecting meagre, Argyrosomus regius, an important aquaculture fish species. Several pathogenic microorganisms have been suggested as causative agents. In this study, we have applied amplicon metagenomics of the 16S rRNA gene in kidneys of fish exhibiting different health states, ranging from apparently healthy to kidneys with calcification. Comparison of the kidney microbiomes of the different fish groups showed that all fish had similar bacterial communities. Linear discriminant analysis effect size revealed only three OTUs significantly enriched in the sick individuals, with no known granuloma-causing species being present among them, reinforcing the hypothesis of a non-infectious cause of the disease in this fish species.

Hepatic sarcoidosis with symptomatic portal hypertension: A report of 12 cases with review of the literature.

Sarcoidosis is a systemic granulomatosis of unknown etiology, characterized by the presence of immune granulomas. Liver damage is a relatively common extra-pulmonary manifestation, occurring in 3.6-30% of cases. Some patients can develop symptomatic portal hypertension (PH). Few series have evaluated the prognosis of symptomatic PH as well as the efficacy and safety of specific treatment on this complication. This is a multicenter retrospective study of cases of histologically proven hepatic sarcoidosis with symptomatic PH (ascites, digestive hemorrhage) and/or hepatic encephalopathy. Demographic characteristics, comorbidities, clinical manifestations of sarcoidosis, biological data, imaging study of the liver, treatment, and clinical outcomes were collected. Twelve patients were identified, with a mean follow-up of 140 months. The M/F ratio was 1 and Caucasian origin was the most represented (75%). Seven patients presented with hepatic comorbidities: metabolic syndrome, chronic alcoholism or chronic viral hepatitis. Apart from hepatic involvement, mediastino-pulmonary involvement was the most common followed by osteoarticular and skin. Liver damage was inaugural in two thirds of cases. Nine patients developed ascites, six presented esophageal varices complicated by gastrointestinal bleeding. Three patients presented with both ascites and variceal bleeding. One case of hepatic encephalopathy was observed. Five patients presented signs of hepatocellular insufficiency during follow-up, of whom three had hepatic comorbidities. Eight out of 12 patients required second-line treatment after failure of corticosteroids, three patients underwent ligation of esophageal varices but with recurrent digestive bleeding in all cases. Two patients benefited from a transjugular intrahepatic portosystemic shunt (TIPS), also with poor result. At the end of follow-up, five patients were alive and seven patients died. Two patients received a liver transplant, with good result and without recurrence of sarcoidosis on the transplant thereafter. Two patients had quiet sarcoidosis on low dose of corticosteroids and one patient was lost to follow-up. Symptomatic PH related to hepatic sarcoidosis is a severe complication, with high morbidity and mortality, and frequent failure of specific treatments of PH. Early management of these patients, with detection of hepatic comorbidities seems important. In case of therapeutic failure, liver transplantation is an option to consider.

A Clinical Case of Generalized Sarcoidosis with a Predominant Lesion of the Spinal Cord

Sarcoidosis, as systemic epithelioid cell granulomatosis, can be accompanied by damage not only to the intrathoracic lymph nodes and lungs, but also to other organs, in particular, the central nervous system and peripheral lymph nodes. In the spectrum of extrathoracic lesions, spinal cord sarcoidosis occurs only in 6-8 % of cases of all brain lesions. The presented clinical example illustrates the lesion of the spinal cord at the level of the thoracic region, although the literature more often describes the lesion of the cervical region. The disease was accompanied by sarcoidosis of the intrathoracic lymph nodes with rapid spontaneous regression and sarcoidosis of the supraclavicular lymph node. The diagnosis was confirmed after a peripheral lymph node biopsy. Spinal cord sarcoidosis in this patient was characterized by rapid regression against the background of parenteral administration of dexamethasone for 14 days, followed by transfer to tablet forms of prednisone. The positive dynamics of spinal cord sarcoidosis refuted the assumption of the presence of a sarcoid reaction in the lymph nodes against the background of a spinal cord tumor. The use of a course of rehabilitation techniques contributed to the recovery of working capacity.

S760 Epidemiology of Glomerulonephritis as an Extraintestinal Manifestation of Inflammatory Bowel Disease: A Large Population-Based Study

Introduction: Inflammatory bowel disease (IBD), including both Crohn’s disease (CD) and Ulcerative colitis (UC), are known to cause many extraintestinal manifestations. There is limited data describing the association between IBD and renal manifestations. This study provides epidemiologic data to further understand the association between IBD and glomerulonephritis (GN). Methods: The aim of this study was to investigate if IBD is associated with GN. Data was collected from a commercial database (Explorys Inc, Cleveland, OH), an aggregate of EHR data from 27 integrated healthcare systems in the US between 1/2017-1/2022. We identified patients with CD and UC based on Systemized Nomenclature of Medicine – Clinical Terms. We compared the prevalence of GN at least 30 days post-CD or post-UC diagnosis to a control cohort without CD or UC. We excluded patients with a diagnostic predisposition for development of GN (Table). Results: Of the 34,063,760 patients, we identified 149,630 cases of CD and 127,300 cases of UC. There were 310 and 260 cases of glomerulonephritis at least 30 days after diagnosis of CD and UC, respectively. Overall prevalence of glomerulonephritis for CD and UC was 207/100000 persons and 203/100000 persons, whereas for the cohort without CD and UC was 72/100000 persons and 72/100000 persons. Prevalence ratios (PR) of developing glomerulonephritis after at least 30 days of a CD and UC diagnosis were 2.88 (95% CI, 2.57-3.22, p< 0.001) and 2.83 (95% CI, 2.51-3.20, p< 0.001), respectively. Figure shows prevalence and PR in CD and UC sub-categorized by demographics. Membranous GN was the most prevalent (CD 90, UC 90), followed by proliferative (CD 50, UC 50), mesangiocapillary (CD 30, UC20), diffuse (CD 10, UC 0) and focal membranoproliferative (CD 10, UC 10). Conclusion: In this large population-based study, glomerulonephritis was significantly more prevalent in patients with both CD and UC compared to those without IBD. Further studies are needed to understand the pathophysiological mechanism underlying renal involvement.Figure 1.: Prevalence and prevalence ratios of glomerulonephritis after at least 30 days post-CD and post-UC diagnosis. Table 1. - Diagnoses excluded prior to analysis Excluded diagnoses Systemic lupus erythematosus Goodpasture's syndrome Hereditary nephritis Post-infectious GN Granulomatosis with polyangiitis Allergic grandulomatosis angiitis Alport syndrome Human immunodeficiency virus Hepatitic B virus Hepatitis C virus Nephrotic syndrome with membranoproliferative glomerulonephritis Primary pauci-immune necrotizing and crescentic glomerulonephritis Berger's immunoglobulin A or immunoglobulin G nephropathy

Potential Benefits of TNF Targeting Therapy in Blau Syndrome, a NOD2-Associated Systemic Autoinflammatory Granulomatosis.

Blau syndrome is a systemic autoinflammatory granulomatous disease caused by mutations in the nucleotide-binding oligomerization domain 2 (NOD2) gene. NOD2 is an intracellular pathogen recognition receptor. Upon binding to muramyl dipeptide (MDP), NOD2 activates the NF-κB pathway, leading to the upregulation of proinflammatory cytokines. Clinical manifestations of Blau syndrome appear in patients before the age of four. Skin manifestations resolve spontaneously in some cases; however, joint and eye manifestations are progressive, and lead to serious complications, such as joint contracture and blindness. Currently, there is no specific curative treatment for the disease. Administration of high-dose oral steroids can improve clinical manifestations; however, treatments is difficult to maintain due to the severity of the side effects, especially in children. While several new therapies have been reported, including JAK inhibitors, anti-IL-6 and anti-IL-1 therapies, anti-TNF therapy plays a central role in the treatment of Blau syndrome. We recently performed an ex vivo study, using peripheral blood and induced pluripotent stem cells from patients. This study demonstrated that abnormal cytokine expression in macrophages from untreated patients requires IFNγ stimulation, and that anti-TNF treatment corrects the abnormalities associated with Blau syndrome, even in the presence of IFNγ. Therefore, although the molecular mechanisms by which the genetic mutations in NOD2 lead to granuloma formation remain unclear, it is possible that prior exposure to TNFα combined with IFNγ stimulation may provide the impetus for the clinical manifestations of Blau syndrome.

Atypical Griscelli Syndrome Presenting with Immune Dysregulation, Systemic Granulomatosis and Normal Pigment Secondary to A Structural Variant in RAB27A

Griscelli Syndrome Type 2 (GS2) is a rare autosomal recessive disease characterized by organ granulomas, central nervous system inflammation, hemophagocytic lymphohistiocytosis (HLH) and partial albinism. Patients have immune dysregulation secondary to dysfunctions in natural killer (NK) cell and T cell cytotoxicity as a result of poor vesicular transport [1-2]. Typically, patients also have abnormal skin and hair pigmentation from accumulation of melanosome clumps in hair shafts and melanocytes. Homozygous or compound heterozygous pathogenic variants in RAB27A are associated with GS2. However, patients with atypical GS2 harbor a structural rearrangement in RAB27A not detected by typical genetic sequencing [3]. We present two cases of atypical GS2 (below and in supplement) characterized by systemic granulomatosis, neuroinflammation and normal skin and hair pigment, secondary to a structural variant (SV) in RAB27A and a second pathogenic sequence variant on the other allele of the gene.

Systemic Granulomatosis in the Context of Solid Tumors: A Study of 10 Cases

Introduction: The association between solid cancers and sarcoidosis is well-known, however,we shouldn’t quickly link a granulomatous lesion to sarcoidosis in a patient suffering from a solid tumor, hence, a granuloma in the context of a solid cancer presents a diagnostic and a therapeutic challenge.&#x0D; Methods: It’s a retrospective monocentric study of 10 cases, suffering from solid tumors, with granulomatous lesions. The type of cancer, the temporal relation between the granulomatous lesion and the cancer, the associated symptoms, the final diagnosis and the disease progression are reported. The patients were separated into three sub groups based on the time of appearance of the granulomatous lesions in relation to the solid tumor.&#x0D; Results: Discovery of granulomatous lesions preceded the diagnosis of cancer in 1 case, discovered concomitantly with cancer in 5 cases and finally after the cancer in 4 cases. The main solid cancer in our study was breast invasive ductal carcinoma (40%), the other cancers were: squamous cell carcinoma of the breast, colorectal adenocarcinoma, ovarian epithelial cancer, esophageal squamous cell carcinoma, thyroid papillary carcinoma and uterine sarcoma.&#x0D; Conclusion: A granuloma in the course of solid cancers maybe associated with multiple etiologies. Therefore, the clinician may face many difficulties, ranging from failing to diagnose a cancer to a wrong conclusion of treatment failure or a relapse. The biopsy is still the only guiding element.

209 TNF-Targeting Treatment Corrects Proinflammatory Signatures of Autoinflammatory Blau Syndrome

Blau syndrome is systemic granulomatosis associated with gain-of-function mutations in NOD2. Clinical symptoms appear before the age of 4 years and mainly affect the skin, joints, and eyes. While treatments with anti-TNF agents have been reported effective, the underlying molecular mechanisms remain unclear. To elucidate the mechanisms of autoinflammation in Blau syndrome and to clarify how TNF-targeting treatment controls the disease phenotype at the cellular level, macrophages were differentiated from peripheral monocytes of 7 patients with Blau syndrome, among them 4 patients received anti-TNF, and global transcriptional profiles were analyzed.

Localized versus systemic granulomatosis with polyangiitis: data from the French Vasculitis Study Group Registry.

To describe the main features at diagnosis and evolution over time of patients with localized granulomatosis with polyangiitis (L-GPA) compared with those of systemic GPA (S-GPA). EULAR definitions of L-GPA, i.e. upper and/or lower respiratory tract involvement, and S-GPA were applied to patients from the French Vasculitis Study Group Registry. L-GPA and S-GPA patients' characteristics at diagnosis and long-term outcomes were analysed and compared. Among the 795 Registry patients, 79 (10%) had L-GPA. Their main clinical manifestations were rhinitis, lung nodules, sinusitis and otitis. L-GPA vs S-GPA patients at diagnosis, respectively, were younger, more frequently had saddle nose deformity or subglottic stenosis and were less often PR3-ANCA-positive. L-GPA vs S-GPA induction therapy less frequently included CYC but more often a combination of MTX and glucocorticoids; 64% of MTX-treated patients experienced disease progression within 18 months post-diagnosis. L- and S-GPA patients' estimated relapse-free-survival probabilities, relapse rates and refractory disease rates at each time point were comparable, but L-GPA patients had more frequent ENT and lung relapses, and higher overall survival rates (P<0.02). Over a median follow-up of 3.5 years, 18 (22.8%) L-GPA progressed to S-GPA, either as a relapse after a period in remission or more frequently in the context of refractory disease. L-GPA patients experienced more ENT-related damage. The relapse risks of L-GPA and S-GPA were similar, but relapse patterns differed and L-GPA overall survival rate was higher. About one-quarter of L-GPA patients developed S-GPA over time, but without end-stage organ involvement.

Renal involvement in sarcoidosis

Sarcoidosis is a systemic granulomatosis that affects the lungs and mediastinum. Renal involvement is rare in this disease. It's most commonly granulomatous interstitial nephritis. The most common form of presentation is naked renal failure. This is a retrospective study that collected 12 cases of sarcoidosis complicated by acute renal failure. The study consisted of identifying the epidemiological, clinical, biological and histologic data that confirmed the diagnosis from January 1994 to January 2016. A search for extra-renal placement of sarcoidosis concerned all cases. Then, a study of the therapeutic and evolutionary profile was carried out in all cases. Twelve patients (8 males and 4 females) with a mean age of 43 years. The intradermal tuberculin reaction sit negative in 6 cases. Biological analysis showed acute renal failure with a median creatinine of 54.32 mg/L, biological inflammatory syndrome in all cases, normal blood calcium in 9 cases, hypercalcaemia in 6 cases, hypercalciuria in 4 cases, hypergammaglobulinemia of polyclonal appearance in 3 cases and elevated proteinuria in 2 cases. Renal ultrasound was normal in all patients. The renal biopsy performed in all cases showed tubulointerstitial nephropathy. The extra-renal signs found were pulmonary interstitial syndrome in 4 cases, dry syndrome in 4 cases, mediastinal lymphadenopathy in 4 cases, one patient presented a unilateral uveitis. Two patients underwent extra-renal treatment. All patients were on corticosteroids with a clear improvement in creatinine and normalisation of renal function in all patients. The average follow-up time was 18 months. Renal involvement during sarcoidosis is rare (10%) but may progress to renal failure (3% of cases). The renal biopsy is of great interest to maintain the diagnosis of sarcoidosis, especially in the absence of other extra-renal signs. Renal involvement during sarcoidosis is rarely revealing and may delay diagnosis, leading to chronic and even end-stage renal disease.

Vitamin D supply in patients with rheumatic diseases in Poland - a pilot study.

ObjectivesIn rheumatic diseases, vitamin D supply is recommended as part of the prophylaxis and treatment of osteoporosis, especially in patients undergoing glucocorticoid therapy, but also due to its immunoregulatory and anti-inflammatory properties. We aimed to evaluate serum 25-hydroxyvitamin D [25(OH)D3] levels in Polish patients with systemic sclerosis (SSc), systemic lupus erythematosus (SLE) and granulomatosis with polyangiitis (GPA), in relation to various clinical parameters, and to assess the initial range of doses for the purpose of further research.Material and methods112 patients (39 with SLE, 44 with SSc and 29 with GPA), referred to the Department of Rheumatology and Internal Medicine in Poznan, Poland, were enrolled in this retrospective study. Demographic and clinical data were collected, including 25(OH)D3 serum levels, vitamin D supplementation doses and season of blood sampling.ResultsMean (SD) serum 25(OH)D3 concentrations were 31 (19.4) ng/ml for SLE, 28.8 (12.5) ng/ml for SSc and 28 (15.2) ng/ml for GPA, and they did not significantly differ between the groups. Vitamin D levels below the optimal range were found in 43.8% of SLE, 65.9% of SSc and 72.4% of GPA patients. 80% of patients reported vitamin D intake, with a mean daily dose of 1398 IU for SLE, 1345 IU for SSc and 1689 IU for GPA. Vitamin D insufficiency and deficiency were frequent among patients with rheumatic diseases, independently of the diagnosis and season.ConclusionsPatients with rheumatic diseases seem to require higher doses of vitamin D than recommended for the general population. The present results indicate the necessity to use higher initial doses of vitamin D in this group of patients (2000 to 4000 UI) and to maintain the dose of vitamin D regardless of the change of seasons.