Polyarthritis Nodosa Research Articles

E37 Clinical and demographic criteria of Juvenile primary systemic vasculitis in Libyan Children

Abstract Introduction Primary vasculitis syndromes are rare in childhood. The clinical manifestations depend on the vessels involved and can lead to significant mortality and morbidity. Aim To study demographic and clinical characteristics of patients with diagnosed with vasculitis other than Kawazaki disease and Henoch-Schonlein purpora (HSP) and to report the outcome of the patients. Materials and methods This is a case series conducted by reviewing the files of patients diagnosed with vasculitis and followed up at the pediatric rheumatology unit of the Tripoli Children Hospital, Libya from year 2000–2022. Data regarding patients’ demography, time of diagnosis, clinical features, laboratory results, treatment and complications were retrieved. Results A total of 37 cases included in the study. 17 (45.9%) were males and 20 (54%) were females followed up for a mean period of 4.48 ± 4.21. Mean age at diagnosis was 8.39 ± 3.84 years. Regarding the diagnosis, there were 3 (8.1%) patients with Takayasu arteritis, 5 (13.5%) patients with polyarteritis nodosa, 2 (5.4%) patients with cutaneous polyarthritis nodosa, 1 (2.7%) patients with granulomatosis with polyahgitis, 1 (2.7%) patient with microscopic polyangiitis, 3 (8.1%) patients with Chrug-Strauss disease, 16 (43.2%) patients with Behcet disease, 1 (2.7%) patients with cogan syndrome, 3(8.1%) patients with urticarial vasculitis, 1 (2.7%) patient with hypocomplementaemic vasculitis, and 1 (2.7%) patient with DADA2. Mean period between the start of symptoms and diagnosis was 1.4 ± 2.8 years. In 6 (16.2%) of the patients there was family history of vasculitis and in 11 (29.7%) the parents are consanguineous. Angiography revealed abnormalities in 5 (13.5%) of the patients. Skin biopsy was done in 4 (10.8%) patients [2 patients with polyarthritis nodosa and 2 patients with urticarial vasculitis] and renal biopsy was done in 1 (2.7%) patient. Steroid was used in 24 (64.9%), Azathioprine in 4 (10.8%) patients. Cellcept was used in 10 (27%) of the patients. Ciclosporin was used 1(2.7%) patient, cyclophosphamide in 3 (8.1%) patients and Immunoglobulin in 6 (16.2%) of patients. Biologics were used in six patients, adalimumab in one patient which was discontinued because of recurrent abscess. In the two patients who used infliximab, treatment was effective. Three patients were treated with tocilizumab, it was ineffective in two, therefore discontinued. However, tocilizumab was effective in one patient with Takayasu arteritis. Complications developed in 23 (62.2%). Muscle atrophy in 1, deforming arthritis in 1, osteoporosis in 1, mouth ulcers in 2, hypertension in 2, hearing loss in 1, chronic sinusitis in 1, cardiovascular accident in 1, cataract in 1, visual impairment in 3, proteinuria in 2, pulmonary fibrosis in 1, chronic asthma in 3, impaired renal function in 1. Conclusion This is the first report of pediatric vasculitis cases in Libya. It showed a heterogeneous spectrum of vasculitis with high rate of consanguinity and low rate of reported family history of vasculitis. Hopefully this report will increase awareness among clinicians of vasculitis and more cases will be diagnosed.

Sjögren's syndrome.

Sjögren's syndrome (SS) is characterised as keratoconjunctivitis sicca (dry eyes), xerostomia (dry mouth) commonly associated with salivary gland enlargement, and is referred to as Primary Sjögren's syndrome. It is known as Secondary Sjögren's syndrome when it occurs in patients, with connective tissue disease, such as rheumatoid arthritis, systemic lupus erythematosus, polyarthritis nodosa, polymyositis, and systemic sclerosis. SS has also been associated with chronic graft-versus-host disease after allogeneic bone marrow transplantation, human immunodeficiency syndrome (AIDS), hepatitis C infection (HCV), chronic biliary cirrhosis, neoplastic and myeloplastic syndromes, fibromyalgia, and chronic fatigue syndrome.

A rare case of spontaneous perirenal hemorrhage - Wunderlich syndrome

Introduction. Spontaneous perirenal hemorrhage or Wunderlich syndrome represents a rare entity in urological settings. The vast majority of the causes are represented by angiomyolipoma and renal cell carcinoma. In other cases, vascular abnormalities, polycystic kidneys, polyarthritis nodosa, or pyelonephritis could represent the cause of perirenal bleeding. The treatment depends on the clinical parameters at the presentation as well as on the possible presence of renal malignancies. Our goal was to present a rare case of a healthy men who presented the idiopathic Wunderlich syndrome. Case outline. We present a case of a 50-year-old patient with spontaneous perirenal hemorrhage that was not due to any defined cause even after a six-months follow-up. Conclusion. In some rare cases of perirenal bleeding, the cause cannot be defined at the presentation, even with today?s advanced radiological imaging methods. Thus, it is important to be aware of the fact that in those cases, a longer follow-up is needed, knowing that the presence of perirenal hematoma can always obscure the real diagnosis. Sometimes, even in cases where the proper follow-up has been done, the real cause of the bleeding remains unknown.

Recent news in the treatment of hepatitis B virus-related cryogobulinemic vasculitis.

Hepatitis B virus (HBV) is a hepatotropic virus that causes hepatitis, cirrhosis and hepatocellular carcinoma. Twenty percent of HBV patients may develop extra-hepatic manifestations, such as polyarthritis nodosa, glomerulonephritis, dermatitis, poly-arthralgia and arthritis, and aplastic anemia. The association of HBV and cryoglobulinemic vasculitis (CV) has been highlighted by several epidemiological investigations. CV can develop in 0.5-4% of HBV infected patients. It has been demonstrated that suppression of HBV replication by nucleot(s)ide analogues (NAs) effectively induces clinical response in most patients with mild to moderate CV, but low responses are seen in severe CV. Based on this evidence, NAs therapy represents the first line therapeutic option in subjects with mild or moderate HBV related CV. Peg-interferon-Alfa can be an alternative treatment for HBV related CV, but the few studies published so far have shown no encouraging results. In patients with severe vasculitis and/or skin ulcers, peripheral neuropathy and glomerulonephritis treatment with rituximab (RTX) and NAs should be considered as a first line treatment. The long-term administration of low-medium glucocorticoid doses has been widely used in few studies to control clinical symptoms, but it should be used as a second option, when RTX is ineffective or not tolerated and in association with NAs. This review focuses on novel treatments for HBV related CV.

STUDY OF PREVALENCE AND PATTERN OF HEARING LOSS IN PATIENTS OF HEPATITIS B

Hepatitis B has been documented to cause various extra hepatic manifestations along with known hepatic complications. It has been reported that hepatitis-B patients are more susceptible to inner ear damage and hearing loss. The aim of this study is to evaluate hearing loss among patients of hepatitis B {all 6 categories Hepatitis B infection: chronic Hepatitis B infection , hepatitis B cirrhosis ,Hepatitis B virus carriers , occult chronic Hepatitis B and Hepatitis B infection with poly arthritis nodosa, hepato cellular carcinoma with hepatitis B}compared with healthy subjects.
 METHOD: In this case control study 100 Hepatitis B positive patients and 100 age and gender-matched healthy individuals were included over the period of 5 years. All of them were known cases of chronic hepatitis B positive for HBsAg at least for 18 months. All patients were aged 18 to 50 years to exclude presence of presbycusis. After base line investigations, they were subjected for all cases and controls were subjected otoscopic examination and hearing assessment using standard pure tone audiometry. Descriptive statistical analysis has been carried out in this study.
 RESULT: In patients of Hepatitis B (94 patients,6 patients had of natural death ) pure tone average (mean thresholds 250,500, 1000,2000,4000 &8000 Hz) was 28.4 dB in the right ear and 27.3 dB in the left (hearing loss).In the control group(96 patients,4 patients dropped out), PTA average was 9.9 dB in the right ear and 9.3dB in the left (normal hearing). In both groups, Speech Discrimination score (SDS) was100% in both ears. The percentage of hearing loss in the right and left ear over the total of six frequencies differed significantly in the two groups. Out of 94 patients of control group, 38 patients (40.4%) patients presented with Chronic Liver Disease (CLD), 14 patients (14.8%) patients presented with cirrhosis with Hepatitis B, 6 (6.3%) patients had Poly arthritis Nodosa with Hep-B, 18(19.1%) patients were diagnosed as carrier of Hepatitis-B , 11(11.7%) patients had occult Hepatitis-B and 7(7.4%) patients were diagnosed with hepato cellular carcinoma.
 Hearing loss was maximum in patients of PAN with Hep-B. Second highest mean SNHL was seen in patients of Hep-B with cirrhosis .Third highest mean hearing loss was noted in patients with HCC .Forth highest mean hearing loss was noted in patients with occult Hep-B. Fifth highest mean hearing loss was noted in carriers of Hep-B.Lowest group with SNHL was chronic liver disease.
 CONCLUSION: Regular audiometric tests are recommended for patients with HBV infection to assess their hearing ability and enable the earlier detection of SNHL. We also suggest that HBV presenting with the sudden onset of hearing loss should be examined for the possibility of acute exacerbation of chronic HBV infection.
 KEYWORDS: Mean, Sensorineural, Hearing loss, Cirrhosis.

Isolated Right Testicular PAN (Polyarthritis Nodosa) presenting as Right Testicular Pain

Patient is a 30 year old male with no significant past medical history who repeatedly sought medical attention for debilitating right testicular pain. Initially he was symptomatically treated with non-steroidal anti-inflammatory medications for presumptive diagnosis of testicular trauma. A few months later he went to different emergency department and was treated with antibiotics for presumptive diagnosis of epididymitis. Finally at his third presentation he was hospitalized. Three testicular biopsies were taken and only the biopsy sample from the middle area of the right testes revealed polyarthritis nodosa. The Patient had no other signs and symptoms and this was likely isolated PAN of his right testes which is a very rare occurrence.

Association Between Hepatitis B and Hearing Status

Viral diseases are one of the common causes of hearing loss. The inner ear may be involved directly or by secondary reaction (e.g., polyarthritis nodosa). This study was performed to investigate the relation between positive HBS-Ag (hepatitis B disease) and hearing loss. This case-study research was done on 95 hepatitis-B patients as the case group and 97 normal cases as the control group. They were selected sequentially and audiologic tests were performed on the participants. The hearing thresholds of the two groups were compared using the t-test. According to audiometry results, pure tone average (mean thresholds of 500, 1000 and 2000 Hz) were 22.1 dB for the left ear and 23.95 dB for the right ear in hepatitis-B group and 8.4 dB for the left ear and 8.95 dB for the right ear in the control group (HBS-Ag negative). The difference between two groups was statistically significant with p-value less than 0.05. The results show that hepatitis-B patients are more prone to hearing loss and that hepatitis B disease can cause hearing loss. This study suggests that hepatitis B prophylaxis is important in decreasing hepatitis-B involvement and therefore, hearing loss.

Shared genetic factors may not explain the raised risk of comorbid inflammatory diseases in multiple sclerosis

Background: Comorbid inflammatory conditions in multiple sclerosis (MS) patients suggest shared risks with MS. Objective: To estimate if the risk of immune-mediated disease in MS patients and their parents is increased. Methods: Swedish register data were analysed using Cox regression to estimate immune-mediated disease risk among 11284 fathers and 12006 mothers of MS patients, compared with 123158 fathers and 129409 mothers of index subjects without MS. Similar analyses were conducted among 20276 index subjects with MS and 203951 without. Results: Parents of patients with MS did not have a significantly altered immune-mediated disease risk. Patients with MS had a consistently raised risk for several immune-mediated diseases: ulcerative colitis, Crohn’s disease, type 1 diabetes, psoriasis, polyarthritis nodosa and pemphigoid. The risk was more pronounced for diseases diagnosed subsequent to MS onset. Conclusion: The increased occurrence of other immune-mediated diseases in MS patients may not be due to shared genetic factors and surveillance bias is likely to be the main or possibly the entire explanation. If not entirely explained by surveillance bias, a modestly raised occurrence of comorbid diseases may be due to shared environmental risks or factors related to MS disease characteristics.

Association between Hepatitis B and Hearing Status

Objective: Viral diseases are one of the common causes of hearing loss. Inner ear may be involved directly or by secondary reaction (eg, poly arthritis nodosa). This goal was to investigate the relation between positive HBS-Ag (hepatitis B disease) and hearing loss. Method: This case-study research was done on 95 hepatitis B patients as case group and 97 normal cases as control group. They were selected sequentially, and audiologic tests were performed on them. The hearing thresholds of the 2 groups were compared using t test. Results: According to our audiometry results, pure tone average (mean thresholds of 500, 1000, and 2000 Hz) were 22.1 dB for left ear and 23.95 dB for right ear in the hepatitis B group, and 8.4 dB for left ear and 8.95 dB for right ear in the control group (HBSAg negative). Difference between 2 groups was statistically significant, with P < .05. Conclusion: The results show that hepatitis B patients are more prone to hearing loss. In other words, hepatitis B disease can cause hearing loss. This study suggests that hepatitis B prophylaxis is important in decreasing hepatitis B involvement and, therefore, hearing loss.

Ulcus cruris in differential dermatological diagnosis

SummaryThe leg ulcer is mostly caused by vascular or venous diseases. About 1% of the German population suffer from a leg ulcer. 50–80% of the leg ulcers are caused by chronic venous insufficiency. Besides, there are many other diseases which can develop a leg ulcer. If a leg ulcer does not heal over months of therapy there are many other differential diagnosis possible. The ulcer is only a symptom, not a diagnosis.Following diseases can induce leg ulcer: vasculitis, rheumatoid arthritis, cryoglobulinaemia, livedo vasculitis, polyarthritis nodosa, haematological, neuropathic, infectious and metabolic disorders, necrobiosis lipoidica (diabeticorum), pyoderma gangraenosum, calciphylaxis, arterial hypertension, chemical and physical injuries. The different diseases are discussed. For the diagnosis it is important that a detailed anamnesis, a typical statement, laboratory and histological findings exist. Sometimes the cooperation between several medical disciplines is necessary. Only an exact diagnosis can induce a differentiated therapy and in many cases the leg ulcer heal up. The aim of this paper is a survey of various non venous or vascular reasons for the development of leg ulcer. However the survey is not completed. But the main causes are mentioned.

Lndium-111-Labeled Granulocyte Head Accumulation in Patients with Wegener’s Granulomatosis

Among the symptoms of systemic vasculitis, purulent rhinorrhea with painful sinusitis is thought to be relatively specific to Wegener's granulomatosis (WG). Sixteen patients with rapidly progressive glomerulonephritis (GN), arteritis and extensive crescents in renal biopsy were studied by head indium-111 (111In)-granulocyte scanning. They included 8 WG, 5 microscopic polyarteritis, 2 necrotizing and crescentic GN and 1 classic polyarteritis nodosa. Autologous granulocytes labeled with 12.3 MBq of 111In-oxine were administered intravenously. Scintigraphic studies were performed at 4 and 24 h post-injection. Compared to the non-WG cases, considered as a whole, significant accumulation of tracer in sinuses was observed in WG patients (Fisher's p = 0.02). Substantial scintigraphic amelioration was obtained in a WG case treated with methylprednisolone pulses and, in another WG case, after high doses of intravenous gamma-globulins. The complete disappearance of facial uptake was obtained after 2 months of intensive therapy (i.e., steroid, cyclophosphamide and plasma exchange) in another WG patient. 111In-oxine granulocyte imaging may be useful in clinical practice as an additional marker of disease activity and a tool of identification of upper respiratory tract involvement.