Recurrent and Extensive Idiopathic Granulomatous Ureteritis: A Localized Hyperimmune Disease with Genetic Predisposition

Idiopathic granulomatous ureteritis (IGU) is a rare autoimmune disorder. Multiple case reports led to defining its clinicopathological inclusion criteria in 1997. Surgical resection and primary reanastomosis, of such pseudotumor, were considered its definitive management and a 4-months corticosteroid-therapy was used once for persistent ureteric lesion despite of 3-months stenting. Long-term follow-up of such disease is limited and management of its extensive and recurrent disease is lacking. In our case report, a 47-year-man had history of a biopsy-proven IGU 4 years ago that was treated with resection and ureteral reimplantation in a cystoplastic (augmented) bladder. Moreover, he had received Corticosteroids and Azathioprine for a total of 2 years to avoid recurrence. Two years later, he presented with recurrent abdominal pains, urinary tract infections and ultimately;bladder neck disease. Cystoscopic examination revealed extensive bladder masses and severe left ureteric stricture. Biopsy of the bladder lesions confirmed the idiopathic granulomatous disease. He improved, with immunosuppressive therapy that included 3 months of Corticosteroids and Mycophenolate mofetil followed by maintenance therapy with Mycophenolate mofetil. Previous animal studies have shown local hyperimmune response with malformation of the transitional epithelium in a genetically predisposed mice indicating genetic predisposition with immune-mediated expression. Hence, in our patient, we proposed long-term immunosuppressive therapy and follow-up. In conclusion;our case report confirms the autoimmune etiology of such disorder and provides new line of management of its extensive and recurrent variant.

Granulomatous mastitis in a 50-year-old male:A case report and review of literature

BACKGROUND Granulomatous mastitis(GM)an inflammatory disease of the breast that usually affects women of childbearing age,occurs very rarely in males.CASE SUMMARY We present a case study of a 50-year-old male patient with GM.The patient developed a breast lump following the cleaning of a previously embedded dirtfilled nipple.While an initial improvement was noted with antibiotic therapy,a recurrence occurred a year later,showing resistance to the previously effective antibiotics.Subsequently,the lesion was excised.The histopathological examination confirmed the diagnosis of GM.CONCLUSION GM should be considered a possible diagnosis of male breast masses.

Primary biliary cholangitis presenting with granulomatous lung disease misdiagnosed as lung cancer:A case report

BACKGROUND There are few cases of pulmonary granulomatous changes secondary to primary biliary cirrhosis(PBC).No case of granulomatous lung disease secondary to PBC misdiagnosed as lung cancer had been reported.CASE SUMMARY A middle-aged woman presented with lung nodules and was misdiagnosed with lung cancer by positron emission tomography/computed tomography.She underwent left lobectomy,and the pathology of the nodules showed granulomatous inflammation,which was then treated with antibiotics.However,a new nodule appeared.Further investigation with lung biopsy and liver serology led to the diagnosis of PBC,and chest computed tomography indicated significant reduction in the pulmonary nodule by treatment with methylprednisolone and ursodeoxycholic acid.CONCLUSION Diagnosis of pulmonary nodules requires integrating various clinical data to avoid unnecessary pulmonary lobectomy.

Granulomatous lobular mastitis treated by a combined internal and external treatment of traditional Chinese medicine:A case report

BACKGROUND Granulomatous lobular mastitis(GLM)is a rare benign inflammatory disease of the breast and is classified under comedo mastitis in traditional Chinese medicine(TCM).The etiology of this disease is unknown,and it mainly occurs in women of childbearing age.The diagnosis depends on histopathological biopsy.At present,there is no systematic and standardized treatment plan for GLM.In the absence of evidence supporting an infectious etiology,affected patients might continue to receive multiple courses of antibiotics and unnecessary surgery.CASE SUMMARY A 37-year-old Chinese woman with a history of coronavirus disease 2019 infection presented with swelling and pain in the left breast.She also had erythema,nodules in the lower extremities,arthritis in both knees,cough,and headache.In the early stage of GLM,the mass was not significantly reduced by conservative treatment with internal application of TCM;hence,surgical treatment was carried out.The aim of postoperative treatment was to drain the pus,eliminate the necrosed tissue,and expand the muscles;fumigation and washing using TCM was applied.CONCLUSION Combined internal and external treatment with TCM,following the principle of“Prioritize internal treatment before ulceration and emphasize external treatment after ulceration”was effective in our patient with GLM.The prognosis was good.We believe that TCM offered valuable therapeutic benefits in this disease.

Correlation Between Granulomatous Mastitis and Autoimmune Function and the Immuneregulating Role of Traditional Chinese Medicine

Granulomatous mastitis(GM)is a benign granulomatous condition,and its pathogenesis may be related to autoimmune disorders.Cellular immunity,humoral immunity,immunoglobulins,and complement could all play a role in the disease process,showing certain clinical patterns.Corticosteroids can quickly control disease progression,and immunosuppressants can be used for complex and refractory GM cases.In traditional Chinese medicine(TCM),“healthy qi”is similar to immune system function.For GM with deficient healthy qi,TCM treatments such as internal and external herbal applications can help regulate immune function and shorten disease duration by staged and TCM treatment,regulating viscera,reinforcing healthy qi,and eliminating pathogenic factors.

Management of granulomatous lobular mastitis: an international multidisciplinary consensus(2021 edition)

Granulomatous lobular mastitis(GLM) is a rare and chronic benign inflammatory disease of the breast. Difficulties exist in the management of GLM for many front-line surgeons and medical specialists who care for patients with inflammatory disorders of the breast. This consensus is summarized to establish evidence-based recommendations for the management of GLM. Literature was reviewed using PubMed from January 1, 1971 to July 31, 2020. Sixty-six international experienced multidisciplinary experts from 11 countries or regions were invited to review the evidence.Levels of evidence were determined using the American College of Physicians grading system, and recommendations were discussed until consensus. Experts discussed and concluded 30 recommendations on historical definitions,etiology and predisposing factors, diagnosis criteria, treatment, clinical stages, relapse and recurrence of GLM. GLM was recommended as a widely accepted definition. In addition, this consensus introduced a new clinical stages and management algorithm for GLM to provide individual treatment strategies. In conclusion, diagnosis of GLM depends on a combination of history, clinical manifestations, imaging examinations, laboratory examinations and pathology.The approach to treatment of GLM should be applied according to the different clinical stage of GLM. This evidencebased consensus would be valuable to assist front-line surgeons and medical specialists in the optimal management of GLM.

Aetiology of idiopathic granulomatous mastitis

Idiopathic granulomatous mastitis is a rare chronic inflammatory lesion of the breast that can clinically and radiographically mimic breast carcinoma. The most common clinical presentation is an unilateral, discrete breast mass, nipple retraction and even a sinus formation often associated with an inflammation of the overlying skin. The etiology of idiopathic granulomatous mastitis is still obscure. Its treatment remains controversial. The cause may be the autoimmune process, infection, a chemical reaction associated with oral contraceptive pills, or even lactation. Various factors, including hormonal imbalance, autoimmunity, unknown microbiological agents, smoking and α 1-antitrypsin deficiency have been suggested to play a role in disease aetiology. In this review, causing factors in the aetiology of idiopathic granulomatous mastitis are reviewed in detail.

Granulomatous lobular mastitis secondary to Mycobacterium fortuitum

Granulomatous lobular mastitis is a rare inflammatory disease of the breast of unknown etiology. Most present as breast masses in women of child-bearing age. A 29-year-old female presented with a swollen, firm and tender right breast, initially misdiagnosed as mastitis. Core needle biopsy revealed findings consistent with granulomatous lobular mastitis, and cultures were all negative for an infectious etiology. She was started on steroid therapy to which she initially responded well. A few weeks later she deteriorated and was found to have multiple breast abscesses. She underwent operative drainage and cultures grew Mycobacterium fortuitum. Granulomatous lobular mastitis is a rare inflammatory disease of the breast. The definitive diagnose entails a biopsy. Other causes of chronic or granulomatous mastitis should be ruled out, including atypical or rare bacteria such as Mycobacterium fortuitum. This is the first reported case of granulomatous mastitis secondary to Mycobacterium fortuitum. With pathologic confirmation of granulomatous mastitis, an infectious etiology must be ruled out. Atypical bacteria such as Mycobacterium fortuitum may not readily grow on cultures, as with our case. Medical management is appropriate, with surgical excision reserved for refractory cases or for drainage of abscesses.

Relationship between granulomatous lobular mastitis and methylene tetrahydrofolate reductase gene polymorphism

BACKGROUND Variations in the methylene tetrahydrofolate reductase(MTHFR)gene have been reported as risk factors for numerous conditions,including cardiovascular disease,thrombophilia,stroke,hypertension and pregnancy-related complications.Moreover,it was reported there is an association between breast cancer and mutations in MTHFR-C677T.However,whether there is an association between MTHFR gene polymorphism and granulomatous lobular mastitis or not has been rarely investigated.AIM To analyze the association between MTHFR gene polymorphism and granulomatous lobular mastitis.METHODS Fifty-one patients with granulomatous lobular mastitis admitted to The First Hospital of Kunming were selected as study samples.Their hospitalization time ranged from February 2018 to February 2019.The 51 patients were included in the experimental group,and another 51 women who underwent physical examination at The First Hospital of Kunming in the same period were included in the control group.Deoxyribonucleic acid and MTFR genetic polymorphism testing were performed in each group.The association between MTHFR gene polymorphism and granulomatous lobular mastitis was observed.RESULTS There were significant differences in genotype frequency and allele frequency of C/C and C/T between the experimental group and the control group(all P<0.05).However,there was no significant difference in frequency of T/T genotype between the two groups(P>0.05).In addition,there was no significant difference in genotype frequency and allele frequency of A/A,A/C and C/C between the two groups(P>0.05).CONCLUSION MTHFR gene C677T locus polymorphism is closely related to granulomatous lobular mastitis.

Treatment of bilateral granulomatous lobular mastitis during lactation with traditional Chinese medicine: A case report

BACKGROUND Granulomatous lobular mastitis(GLM)is a type of benign chronic inflammatory disease that poses therapeutic challenges to healthcare providers.The diagnosis of GLM relies on tissue biopsy,and incorrect treatment may lead to delayed diagnosis,considerable aesthetic damage,and even mastectomy.CASE SUMMARY We report the case of a 37-year-old Chinese woman who was lactating and had GLM in both breasts.At the time of treatment,the right breast had a mass of approximately 15 cm×11 cm,which was hard and had poor mobility.Multiple skin ulcerations and pus spills were also observed on the surface of the breast.The left breast had a mass of about 13 cm×9 cm,which was hard and had poor mobility.CONCLUSION Herein,we report a case of bilateral GLM in a lactating woman that was successfully treated with traditional Chinese medicine(TCM),without the requirement for surgery or other treatments.Therefore,TCM may have advantages in the nonsurgical treatment of GLM.

Reversion of severe hepatopulmonary syndrome in a non cirrhotic patient after corticosteroid treatment for granulomatous hepatitis:A case report and review of the literature

Hepatopulmonary syndrome （HPS） is defined as a clinical triad including liver disease, abnormal pulmonary gas exchange and evidence of intrapulmonary vascular dilatations. We report a 61-year-old male presented with fatigue, long-lasting fever, loss of weight, signs of portal hypertension, hepatosplenomegaly, cholestasis and progressive dyspnoea over the last year. Clinical, laboratory and histological findings confirmed the diagnosis of granulomatous hepatitis. HPS due to hepatic granulomainduced portal hypertension was proved to be the cause of severe hypoxemia of the patient as confirmed by contrast-enhanced echocardiography. Reversion of HPS after corticosteroid therapy was confirmed by a new contrastenhanced echocardiography along with the normalization of cholestatic enzymes and improvement of the patient＇ s conditions. This is the first case of complete reversion of HPS in a non-cirrhotic patient with hepatic granuloma, indicating that intrapulmonary shunt in liver diseases is a functional phenomenon and HPS can be developed even in miscellaneous liver involvement as in this case.

Differential diagnosis in ulcerative colitis in an adolescent: Chronic granulomatous disease needs extra attention

Chronic granulomatous disease(CGD) is a primary immune deficiency that is commonly diagnosed under the age of 5 years(95%) and is rarely seen in adulthood. CGD may manifest as inflammatory bowel disease(IBD) in childhood. Without proper diagnosis, these patients may be monitored for years as IBD; some may even be regarded as steroid-resistant ulcerative colitis(UC) and end up having a colectomy. In this case report, we described a patient who had been followedup for years as UC and subsequently underwent colectomy, but was finally diagnosed in adulthood as primary immune deficiency.

Granulomatous Mastitis: Incidence and Experience in a Mexican Institute

The significance of this work is that chronic granulomatous disease can mimic two common breast diseases;breast cancer and breast abscess;so it is mandatory that the diagnosis is made in order to avoid mistaken carcinoma diagnosis. On the other hand, the current treatment of this particular disease still remains controversial. Objectives: The aim of this study is to expose and describe the chronicity and the impact in a patient’s quality of life with Granulomatous Mastitis diagnosis. We describe the evolution of the disease and the treatment of the disease in a Mexican Institution. Material and methods: In a descriptive retrospective study we identified 9 patients with idiopathic granulomatous mastitis (IMG), all of them were diagnosed with histopathological findings. Results: In a period of time between January 2005 and December 2017, we included a total of 9 female patients. All of them have had histopathological findings consistent with granulomatous mastitis. The age range was 25 - 83 years old (mean 50.8 yrs). In 4 patients (44.4%) had the right side affected, three patients (33.3%) had the left side affected and 2 patients (22.2%) had a bilateral presentation. In 3 patients (33.3%) we observed more than one affected quadrant and 4 patients (44.4%) had a diffuse presentation on image. The most common symptom was breast pain in 9 patients (100%) and the most common sign was the presence of a nodule, 8 patients (88.8%). We observed erythema in 7 cases (77.7%) followed by abscess, edema and pus secretion in 6 patients (66.6%). The most frequent treatment was the use of a combination of NAIDs and antibiotic in 8 patients (88.8%). Six patients were incised with scalp and drained. Six patients underwent mastectomy.

Granulomatous Mastitis with Erythema Nodosum That Responded to Low-Dose Steroid: Case Report and Literature Review of Nine Patients

A 31-year-old woman was referred with a painful mass in the left breast that was initially treated with antibiotics. Three weeks later, the mass had increased and polyarthritis and erythema nodosum had developed in both legs. A core needle biopsy led to a diagnosis of granulomatous mastitis with arthritis and erythema nodosum. Low-dose prednisolone (10 mg) rapidly improved the arthritis and the erythema nodosum. Granulomatous mastitis is a very rare, chronic inflammatory disease and only nine patients with granulomatous mastitis with erythema nodosum have been described. We reviewed the literature to determine the clinicopathological features and treatment of granulomatous mastitis. Appropriate diagnosis and steroid therapy should result in excellent outcomes.

An outbreak of granulomatous inflammation associated with Francisella noatunensis subsp, orientalis in farmed tilapia (Oreochromis niloticus × O. aureus) in China

In 2013,a novel disease was detected in tilapia( Oreochromis niloticus × O.aureus) in Guangzhou,South China.To identify the causative pathogen,we conducted histological examination,bacteria isolation,and purification,and sequenced the 16 S rRNA gene of isolates.Infected fish had a large number of white granulomas in the kidney,liver,heart,and spleen.The head kidney and spleen were markedly swollen.A bacterium strain designated as gz201301 was recovered from the spleen of infected tilapia.The 16 S rRNA sequence of gz201301 revealed that it was highly similar to F.noatunensis subsp.orientalis.This is the first report of a Francisella-like infection in farmed tilapia in China.

Etiology of granulomatous inflammation:A retrospective study of 174 children in a tertiary care center

Objective:To investigate granulomatous inflammation etiology based on clinical history and ancillary tests.Methods:Children aged<18 years with biopsy proven granulomatous lesions in any tissue specimens between January 2014 and January 2022 were included in the study.The diagnosis was based on the results of immunohistochemical staining,molecular tests,culture,serology,radiological and other auxiliary laboratory tests.Diagnoses were categorized into infectious and noninfectious causes.Results:In total,174 patients with granulomatosis inflammation confirmed by histopathology were analyzed.Approximately 59.2%patients were males,and the median age was 4.48(IQR 2.36-6.39)years(range:16 months-18 years).The tissues/organs that were most commonly biopsied were lymph node,bone,skin,and lung(51.1%,17.8%,9.2%,and 5.7%,respectively).Infectious and non-infectious causes were identified in 73.0%and 12.6%patients,respectively,in terms of granulomatosis inflammation etiology;however,no cause was identified in 14.4%patients.The most common infectious cause was tuberculosis(in 51.7%patients),followed by toxoplasmosis,aspergillosis,mucormycosis,leishmaniasis,and cat-scratch disease(in 8.6%,5.7%,1.7%,1.7%,and 1.1%patients,respectively).The common non-infectious cause was chronic granulomatous disease.Histopathological evaluation revealed granulomatosis inflammation in 33.3%patients,necrotizing granulomatosis inflammation in 30.5%patients,and caseating granulomatosis inflammation in 12.1%patients.When the pathology results of patients with and without tuberculosis were compared,the incidence of caseating granulomatosis inflammation(P=0.003)and necrotizing granulomatosis inflammation(P=0.005)was higher in patients with tuberculosis.Conclusions:Chronic granulomatous disease is the most common non-infectious cause in children.Moreover,primary or secondary immune deficiencies may cause granulomatosis inflammation,especially in pediatric patients.

Gastrointestinal symptoms as the first sign of chronic granulomatous disease in a neonate: A case report

BACKGROUND Chronic granulomatous disease(CGD)characterized by recurrent and severe bacterial and fungal infections is most common in childhood.CASE SUMMARY We reported a 24-d-old male infant who developed gastrointestinal symptoms as the first sign of CGD.CONCLUSION Gastrointestinal symptoms representing the first sign of CGD are very rare,and prompt diagnosis and treatment with broad-spectrum antibiotics were of crucial importance.

Granulomatous prostatitis after bacille Calmette-Guérin instillation resembles prostate carcinoma:A case report and review of the literature

BACKGROUND Bacille Calmette-Guérin(BCG)instillation is recommended in patients with nonmuscle-invasive bladder cancer who have intermediate-risk and high-risk tumors.However,granulomatous prostatitis is a rare complication induced by BCG instillation,which can easily be misdiagnosed as prostate cancer.Here,we report a case of granulomatous prostatitis that resembled prostate cancer.CASE SUMMARY A 64-year-old Chinese man with bladder cancer received BCG instillation.Three days later,he stopped BCG instillation and received anti-infective therapy due to the urinary tract infection.Three months after BCG restart,he had rising total prostate-specific antigen(PSA)(9.14 ng/mL)and decreasing free PSA/total PSA(0.09).T2-weighted images of magnetic resonance imaging(MRI)showed a 28 mm×20 mm diffuse low signal abnormality in the right peripheral zone,which was markedly hyperintense on high b-value diffusion-weighted MRI and hypointense on apparent diffusion coefficient map images.Considering Prostate Imaging Reporting and Data System score of 5 and possibility of prostate cancer,a prostate biopsy was conducted.Histopathology showed typical features of granulomatous prostatitis.The nucleic acid test for tuberculosis was positive.He was finally diagnosed with BCG-induced granulomatous prostatitis.Thereafter,he stopped BCG instillation and received anti-tuberculosis treatment.During 10 mo follow-up,he had no evidence of tumor recurrence or symptoms of tuberculosis.CONCLUSION Temporarily elevated PSA and high followed by low signal abnormality on diffusion-weighted MRI are important indicators of BCG-induced granulomatous prostatitis.

Surgical outcomes of complicated cataract with pediatric trematode granulomatous uveitis

AIM:To describe the clinical characteristics and surgical outcomes of complicated cataract with pediatric trematodal granulomatous uveitis(TGU).METHODS:Patients of cataract with TGU in the membranous(inactive) stage underwent cataract surgery with intraocular lens(IOL) implantation.Preoperative history and ophthalmic examination were conducted for all cases,whereas Schimphlug imaging and corneal topography were done for some patients.Postoperative follow up was done on the 1st,2nd,and 5th postoperative days after surgery.Then,it was done at least at one,three,and six months postoperatively.Intraoperative and postoperative complications and the methods of their management were reported.RESULTS:Twelve eyes of 12 male children were included in this study ranging from 8 to 16y.The mean best corrected Snellen visual acuity(BCVA) was significantly improved from 0.09±0.06 preoperatively to 0.37±0.11 at the final visit 6mo postoperatively(P<0.001).Schimphlug imaging and corneal topography showed flattening of the anterior surface of the inferior cornea.Intraoperative difficulties and complications included the poor dilatability of the drown down pupil,strong posterior synechia between the anterior lens capsule and the iris at the site of the inferior retrocorneal vascularized membrane and hyphema.All cases underwent primary hydrophobic IOL implantation.CONCLUSION:Surgery for this type of cataract is relatively safe and effective.It is associated with some specific difficulties and complications that should be considered during surgery and follow up.

Granulomatous interstitial lung disease in a long-term drug abuser

It is the habit of some drug consumers to dissolve the powder of crushed pills, intended for oral use, in water and inject this solution intravenously. Insoluble particles than obstruct pulmonary vessels causing microscopic pulmonary emboli. These foreign bodies migrate and penetrate into the perivascular space and interstitium, resulting in chronic inflammation and foreign body giant cell reaction. As a result of this a granulomatous interstitial fibrosis can develop, which has also been described as pulmonary talcosis. We are reporting the case of a 22 year old male with a history of long-term intravenous drug abuse. He presented to our hospital complaining of dyspnoea, cough and generalized weakness. We describe an extensive diagnostic process concluded by an open lung biopsy establishing a definitive diagnosis of this rare granulomatous lung disease. This case underlines the importance of a thorough diagnostic work up and the pathogenic potential of foreign material reaching the lung via blood circulation in amongst the differential diagnoses of interstitial lung diseases, especially occurring in this group of patients.