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California Medicine Oct 1953The collagen diseases, an ill-defined group of clinical entities, have as their basis a generalized alteration of the connective tissue, especially of its extracellular...
The collagen diseases, an ill-defined group of clinical entities, have as their basis a generalized alteration of the connective tissue, especially of its extracellular components. They include periarteritis nodosa, disseminated lupus erythematosus, dermatomyositis, scleroderma, rheumatic fever and rheumatoid arthritis. The radiological findings in a series of cases of these diseases were reviewed. In 28 cases of periarteritis, 20 cases showed some abnormal findings in the thorax. These included pleural effusions, pulmonary changes, pericardial effusions and cardiac enlargement. In 32 cases of disseminated lupus erythematosus, thoracic findings were noted in 21. They resembled the changes found in periarteritis. In some 25 cases of scleroderma, diverse radiological findings were noted. These included "cystic" changes in the lungs (one case) and pulmonary "hives." In the intestinal tract esophageal and small bowel alterations were found, both ectatic and stenotic. In the soft tissues of the "pressure areas" variable degrees of calcification were observed. Dermatomyositis is the rarest of the collagen disease group; only one autopsy-proven case is available for study. Chest x-rays taken a year before death showed slight cardiac enlargement. The lungs were clear. In acute rheumatic fever, x-ray examination may disclose pericardial or pleural effusion, and so-called rheumatic pneumonitis; the latter has no specific diagnostic features. Soft tissue swellings may develop around some of the joints. In rheumatoid arthritis, joint changes are numerous and fairly characteristic, and are followed in many cases by fibrous or bony ankylosis and deformities of considerable degree. Awareness of the commoner radiological changes in this entire group of diseases should result in earlier establishment of diagnosis, especially in the more obscure examples.
Topics: Arthritis, Rheumatoid; Collagen Diseases; Connective Tissue; Dermatomyositis; Humans; Lung; Lupus Erythematosus, Systemic; Pleural Effusion; Pneumonia; Polyarteritis Nodosa; Rheumatic Fever
PubMed: 13094539
DOI: No ID Found -
World Journal of Gastroenterology May 2013The differential diagnosis of acute abdomen is typically extremely broad in range, with vasculitis posing a rare but potentially life-threatening cause of acute abdomen.... (Review)
Review
The differential diagnosis of acute abdomen is typically extremely broad in range, with vasculitis posing a rare but potentially life-threatening cause of acute abdomen. Here, we report a case of acute abdomen with bowel wall thickening limited to jejunum, accompanied by unexplained renal dysfunction. Later, the patient was diagnosed as having polyarteritis nodosa based on surgically resected jejunal necrosis. Despite aggressive treatment, including the use of steroid pulse therapy and continuous hemodiafiltration, the patient died. Although polyarteritis nodosa is extremely rare in patients with acute abdomen, acute abdomen is relatively common manifestation of that. And it is reported that involvement of small intestine suggests poorer prognosis. Our case highlights the importance of vasculitis as a differential diagnosis of patients with atypical acute abdomen. In this report, we not only review possible clues that might have led to an earlier diagnosis in this case, but also attempt to draw some lessons for treating similar cases in the future.
Topics: Abdomen, Acute; Aged; Biopsy; Fatal Outcome; Female; Hemodiafiltration; Humans; Immunosuppressive Agents; Jejunum; Kidney Diseases; Necrosis; Polyarteritis Nodosa; Predictive Value of Tests; Pulse Therapy, Drug; Steroids; Tomography, X-Ray Computed; Treatment Outcome
PubMed: 23687422
DOI: 10.3748/wjg.v19.i18.2830 -
Clinical & Developmental Immunology 2004The best therapeutic strategy in virus-induced vasculitides should take into account the etiology of the disease and be adapted to the pathogenesis. The combination of... (Review)
Review
The best therapeutic strategy in virus-induced vasculitides should take into account the etiology of the disease and be adapted to the pathogenesis. The combination of antiviral treatments and plasma exchanges has been proven effective in polyarteritis nodosa (PAN). In human immunodeficiency virus (HIV)-related vasculitis this strategy is also effective and does not jeopardize, like cytotoxic agents, the outcome of AIDS. In vasculitis related to HCV-associated cryoglobulinemia, plasma exchanges improve the outcome but the poor effectiveness of antiviral drugs is not able to favor, usually, a definite recovery of the patients and relapses are frequent.
Topics: Anti-HIV Agents; Antiviral Agents; Cryoglobulinemia; HIV Infections; Hepatitis B; Hepatitis C; Humans; Interferon Type I; Plasma Exchange; Polyarteritis Nodosa; Recombinant Proteins; Vasculitis; Virus Diseases
PubMed: 15559368
DOI: 10.1080/17402520400001744 -
Internal Medicine (Tokyo, Japan) 2014We herein report the case of a Japanese man with polyarteritis nodosa (PAN) accompanied by multiple myeloma (MM). The patient was diagnosed with PAN. Concurrently, IgG... (Review)
Review
We herein report the case of a Japanese man with polyarteritis nodosa (PAN) accompanied by multiple myeloma (MM). The patient was diagnosed with PAN. Concurrently, IgG kappa paraprotein was detected, and bone marrow changes indicative of MM were observed. Prednisolone (PSL) administered at a dose of 30 mg/day was initiated; however, the serum creatinine level increased. In spite of increasing the dose of PSL to 45 mg/day and initiating treatment with double filtration plasmapheresis, the patient's renal dysfunction continued to progress and haemodialysis was introduced. He died from pneumonia 12 months after admission. We conclude that renal failure is an important risk factor in the prognosis of PAN accompanied by MM.
Topics: Acute Kidney Injury; Fatal Outcome; Humans; Male; Middle Aged; Multiple Myeloma; Polyarteritis Nodosa
PubMed: 24492698
DOI: 10.2169/internalmedicine.53.1120 -
Postgraduate Medical Journal Jun 1953
Topics: Arthritis; Arthritis, Rheumatoid; Humans; Lupus Erythematosus, Systemic; Polyarteritis Nodosa; Tuberculosis; Tuberculosis, Miliary
PubMed: 13055554
DOI: 10.1136/pgmj.29.332.321 -
Journal of Clinical Rheumatology :... Dec 2023
Topics: Humans; Gangrene; Polyarteritis Nodosa
PubMed: 37337335
DOI: 10.1097/RHU.0000000000001998 -
Scientific Reports May 2016Sexual dimorphisms are recognized in cardiovascular conditions such as hypertension, stroke, thrombosis and vasculitis. B-type natriuretic peptide (BNP) is a guanylyl...
Sexual dimorphisms are recognized in cardiovascular conditions such as hypertension, stroke, thrombosis and vasculitis. B-type natriuretic peptide (BNP) is a guanylyl cyclase A (GC-A) agonist. The anti-hypertensive, vasodilatory, anti-fibrotic, and anti-hypertrophic properties of BNP are well established in male animal models. Although circulating BNP levels are higher in women, when compared to age-matched men, the cardiovascular protective propensity of BNP in females is poorly understood. We assessed the cardiovascular consequences of BNP deletion in genetically null (Nppb-/-) female rat lines. Throughout the study, blood pressure (BP) remained uninfluenced by genotype, and cardiorenal consequences of BNP knock out remained minor. Unexpectedly, approximately 60% of Nppb-/- females developed mesenteric polyarteritis-nodosa (PAN)-like vasculitis in their life span, some as early as 4 months of age. Mesenteric lesions involved intense arterial remodeling, progressive inflammation, occluded lumens, and less frequently intestinal necrosis and multiple visceral arterial aneurysms. Cumulative pathologies resulted in a significant decline in survival of the Nppb-/- female. This study highlights BNP's vasoprotective propensity, bringing to light a possible sex specific difference in the cardiovascular protection provided by BNP. Defects in the BNP/GC-A/cGMP pathway may play a role in arteriopathies in women, while GC-A agonists may provide effective therapy for arteritis.
Topics: Animals; Blood Pressure; Female; Humans; Hypertension; Male; Mesenteric Arteries; Natriuretic Peptide, Brain; Polyarteritis Nodosa; Rats, Inbred Dahl; Sex Factors; Time Factors; Vascular Remodeling; Vasculitis
PubMed: 27162120
DOI: 10.1038/srep25623 -
Hinyokika Kiyo. Acta Urologica Japonica Dec 2016This report documents a case of asynchronous bilateral testicular infarction. The patient was a 42- year-old man who presented with left testicular pain and swelling. He... (Review)
Review
This report documents a case of asynchronous bilateral testicular infarction. The patient was a 42- year-old man who presented with left testicular pain and swelling. He had a past history of right idiopathic testicular infarction and underwent a right orchiectomy 6 years ago. He also had received treatment for 5 years for suspected polyarteritis nodosa (PAN). The left scrotal pain persisted for a week and left orchiectomy was performed. Pathological evaluations demonstrated a benign testis with testicular hemorrhage and chronic vasculopathy. There was no fibrinoid necrosis of medium-size vessel walls which characterizes PAN. In this report, we review the pathogenesis, risk of contralateral testicular infarction, and management of testicular infarction.
Topics: Adult; Hemorrhage; Humans; Infarction; Magnetic Resonance Imaging; Male; Multimodal Imaging; Orchiectomy; Pain; Polyarteritis Nodosa; Testicular Diseases; Tomography, X-Ray Computed
PubMed: 28103661
DOI: 10.14989/ActaUrolJap_62_12_657 -
BMJ Case Reports Feb 2022Polyarteritis nodosa (PAN) is a medium vessel vasculitis with necrotising vascular changes along with multisystemic involvement. Due to variable initial presentations,...
Polyarteritis nodosa (PAN) is a medium vessel vasculitis with necrotising vascular changes along with multisystemic involvement. Due to variable initial presentations, diagnosis of systemic PAN in children requires a comprehensive work up. In addition, systemic PAN needs an aggressive therapy. Mycophenolate mofetil is an emerging newer alternative for the treatment of PAN. We report a case of childhood systemic PAN who initially presented with subtle signs like reduced sensation over lateral foot, non-deforming arthritis and multiform rashes. After comprehensive aetiological work up, nerve biopsy and supporting evidence clinched the diagnosis. Vasculitis in children presenting with benign subtle signs is sometimes a diagnostic challenge to clinicians. Our case highlights the importance of lateral thinking while dealing with non-specific multisystemic signs. Evidence of successful treatment of PAN with mycophenolate mofetil is gradually being built up. It is also described to result lower relapse and increased treatment free survival rate.
Topics: Biopsy; Child; Humans; Immunotherapy; Mycophenolic Acid; Polyarteritis Nodosa; Recurrence
PubMed: 35140101
DOI: 10.1136/bcr-2021-248477 -
BMJ Case Reports Mar 2014We describe the case of a previously healthy 12-year-old boy admitted to a tertiary paediatric centre with the clinical diagnosis of Henoch-Schonlein purpura (HSP)....
We describe the case of a previously healthy 12-year-old boy admitted to a tertiary paediatric centre with the clinical diagnosis of Henoch-Schonlein purpura (HSP). Symptoms on admission included a generalised rash, colicky abdominal pain, hypertension, proteinuria and fresh rectal bleeding. Abdominal pain and distension worsened and serial ultrasounds suggested bowel ischaemia. He underwent repeat laparotomy and bowel resection, with slow improvement after the second laparotomy. The severity of systemic involvement (gastrointestinal, cardiac, renal and skin) made the initial diagnosis of HSP questionable. Immunohistochemistry of skin biopsies was negative for HSP. Histopathology of the bowel specimen revealed features of necrotising small and medium vessel vasculitis in keeping with polyarteritis nodosa.
Topics: Child; Diagnosis, Differential; Humans; IgA Vasculitis; Male; Polyarteritis Nodosa
PubMed: 24717855
DOI: 10.1136/bcr-2013-201764