1/12. PFAPA syndrome (Periodic fever, Aphthous stomatitis, pharyngitis, Adenitis).This paper aims to remind paediatric clinicians to suspect and confirm 'PFAPA' syndrome (Periodic fever, Aphthous stomatitis, pharyngitis and cervical Adenitis syndrome). We report two cases of PFAPA syndrome: a 3-year-old healthy boy with atopic rhinitis and a boy aged 8 years 5 months who simultaneously had lymphocytic vasculitis syndrome treated with immunosuppressive drugs. Both met Marshall's criteria. The literature regarding PFAPA syndrome was complied using a medline search for articles published between 1963 and 1998 and we then reviewed the reference lists of the articles. The medline search revealed 28 cases with available clinical manifestations, management and prognosis. Our study describes two additional cases. We divided the cases into typical (28 cases) and atypical (two cases) PFAPA syndrome. In typical PFAPA, the age of onset was less than 5 years in most cases and the patients presented 4.9 /- 1.4 days of fever (100%), pharyngitis (89.3%), cervical adenitis (72.1%), stomatitis (71.4%), malaise (64.3%), headache (60.7%), abdominal pain (53.6%) and nausea/vomiting (17.9%). Afebrile intervals were 3.2 /- 2.4 months and increased with age. The time from initial onset to final episode was 3 years 7 months /- 3 years 6 months. The total number of episodes was 8.3 /- 2.5 (range 6-14). Effective treatment included steroids, tonsillectomy/adenoidectomy and cimetidine. The general outcome was good. In atypical PFAPF, the clinical manifestations were similar to those of typical PFAPA except that the age of onset was more than 5 years, and life-threatening intestinal perforation happened once in a patient with underlying Fanconi's anaemia. It was concluded that typical PFAPA syndrome is benign and can be diagnosed by detailed history-taking and from physical findings during repeated febrile episodes with tests to rule out other periodic fever syndromes. A review of the literatures since the first report in 1987 has shown that typical PFAPA syndrome is not associated with significant long-term sequelae and has a good response to steroids. One patient with atypical PFAPA, who received low-dose steroids for over 1 year, developed intestinal perforation after an increment of the 7-day steroid dose. If an underlying problem requires long-term immunosuppressive medication, it is wiser to choose cimetidine rather than increasing the steroid dosage to resolve atypical PFAPA.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
2/12. Cases from the medical grand rounds of the Osler Medical Service at Johns Hopkins University.A 77-year-old man was in good health until he complained of fatigue 3 weeks before presentation. Two weeks before admission, he developed gradually worsening shortness of breath. One week before admission, he developed a cough that initially was nonproductive but later was associated with hemoptysis.His past medical history was remarkable for a history of colon cancer (Dukes' stage III), for which he underwent a hemicolectomy and treatment with adjuvant chemotherapy in 1993. He had a myocardial infarction in 1986 and underwent coronary artery bypass surgery in 1999. He also had a history of hypertension, type 2 diabetes, and gout. He smoked in the past but had stopped more than 30 years ago.He was initially evaluated by his primary care physician, who noted that he complained of diaphoresis but denied fevers, chills, or contact with others who were ill. His physical examination was remarkable for bilateral crackles that were more pronounced on the right. A chest radiograph demonstrated bilateral pulmonary infiltrates (Figure 1). He was treated with amoxicillin. The next day, however, his physician noted that his dyspnea had worsened and that his oxygen saturation on room air was poor. He was therefore admitted for further evaluation.The amoxicillin was discontinued, and he was treated with levofloxacin, followed by ceftriaxone and azithromycin as his pulmonary status continued to deteriorate. He received intravenous diuretic agents, which failed to improve his respiratory status.During the initial phase of hospitalization, he was anemic, with a hematocrit of 21.3%. His serum creatinine level, which had been 1.0 mg/dL in 1999, was now 2.5 mg/dL. urinalysis was remarkable for the presence of numerous red blood cells. His oxygen requirement increased, and he eventually required a 100% nonrebreather mask. A computed tomographic scan of the chest demonstrated prominent alveolar opacities throughout the right upper, middle, and lower lobes, with similar opacities in the left upper and left lower lobes (Figure 2). An echocardiogram showed an ejection fraction of 50%, as well as mild mitral regurgitation. Serologies were remarkable for an antinuclear antibody titer of 1:320 and a P-antineutrophil cytoplasmic antibody (P-ANCA) titer of greater than 1:320. C-ANCA was negative. Anti-glomerular basement membrane and anti-human immunodeficiency virus antibodies were undetectable.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
3/12. Microvasculitic paraproteinaemic polyneuropathy and B-cell lymphoma.Microvasculitis may play a greater part in the pathogenesis of paraproteinaemic neuropathies than is generally recognised, producing tissue destruction by convergent immune and physical mechanisms. We present a patient with a clinical syndrome of mononeuritis multiplex and a circulating IgM lambda paraprotein, in whom bone marrow aspiration revealed a lymphoplasmacytoid lymphoma. Microvasculitic changes were present in the first nerve biopsy, and the second showed extensive destruction of neural architecture and deposition of IgM-related material. A 2-stage pathogenic cascade is postulated and explored with a review of the relevant literature.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
4/12. Necrotic facial papules in an adolescent: C2 deficiency with eventual development of lupus erythematosus.A 14-year-old girl was admitted to the hospital because of persistent throat pain, fever, fatigue, 25 pound weight loss, and leukopenia. On physical examination she was thin, ill-appearing, and had necrotic papules on the face and palpable cervical lymph nodes. Presumptive differential diagnosis included occult malignancy and infection. Numerous investigative procedures failed to elucidate a source. Vasculitis was eventually appreciated after repeat skin biopsy. Numerous serologic studies were performed and were notable for a very low level of the second component of complement without direct evidence of lupus erythematosus (LE) or other autoimmune conditions. A diagnosis of C2 deficiency-associated vasculitis was made. She was treated with high-dose prednisone and cyclophosphamide with resolution of her symptoms. Two years later she returned with marked malar erythema. Antinuclear and Smith antibodies were then detected and a diagnosis of LE was made. She was treated with hydroxychloroquine and sun-avoidance measures with clearance of the malar rash.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
5/12. Nodular pulmonary vasculitis in a twelve-year-old boy.A 12-year-old boy presented with left shoulder pain during physical exercise and complained of uncommon sweating and fatigue. Diagnostic evaluation revealed a solitary pulmonary nodule in the left upper lobe. All laboratory values were within normal limits, except for an elevated level of antineutrophil cytoplasmic antibodies directed against myeloperoxidase (p-ANCA). Surgery was performed, and pathological examination showed a localized granulomatous vasculitis. Antineutrophil cytoplasmic antibodies directed against affinity purified proteinase 3 (p-ANCA) concentrations returned to baseline within 6 months, and the patient has done well during a follow-up period of 2 years. While nodular vasculitis is known to occur in Wegener's granulomatosis, to the best of our knowledge, this case represents the first c-ANCA negative primary pulmonary vasculitis in childhood.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
6/12. Roadmap to vasculitis.Vasculitis is characterized by vessel wall injury caused by an immunologically initiated inflammatory reaction. Vesselwall injuryleads to vascular stenosis, aneurysm, bleeding, thrombosis, embolism, vasospasms and ischemia. The vasculitis is clinically important when the patient has general inflammatory and multifocal symptoms, which progress in episodes and can be explained by these vascular lesions. The clinical manifestations of these depend on the size, localization and number of blood vessels involved. This forms the basis of the current vasculitis classification. It is important to recognize the secondary vasculitides, as their treatment is mainly based on elimination of the triggering factor. In primary vasculitides, immunosuppression alone is the basis of treatment in almost all cases, whereas the management of pseudovasculitis is dependent on its aetiology. In primary care, basic evaluation should be done: patient history, physical examination, basic laboratory tests and other non-invasive tests to verify suspected surrogate findings. After this, patients should be urgently referred to a specialized centre, where the required histological and radiological tests are performed for diagnosis and immunosuppressive and other necessary treatment is initiated.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
7/12. A case of polymyalgia rheumatica, microscopic polyangiitis, and B-cell lymphoma.BACKGROUND: A 73-year-old, previously well woman was admitted to an emergency department because of a 3-month history of severe proximal girdle pain and stiffness with loss of appetite and weight. She was referred to a rheumatologist 10 days after her initial presentation. Within 4 weeks she presented to an outpatient clinic with nausea, vomiting, shortness of breath, painful mouth ulcers, rash on her legs and a further decline in appetite; she was readmitted to hospital. Within 4 months of initial presentation she became jaundiced. INVESTIGATIONS: At initial presentation, physical examination, biochemical, hematological and autoimmune screening, radiography of the pelvis, an abdominal ultrasound, and electromyography were conducted. At referral to a rheumatologist similar tests were repeated. At presentation to the outpatient clinic, hematological and biochemical screening, and a urine dipstick test were conducted. At readmittance to hospital, infectious and autoimmune screening, radiography of the chest, electrocardiogram, ultrasound of the abdomen, and renal biopsy were conducted. At the time of development of jaundice, biochemical and hematological screening, CT of the abdomen and ultrasound-guided biopsy of a pancreatic mass were conducted. diagnosis: polymyalgia rheumatica, antineutrophil-cytoplasmic-antibody-positive microscopic polyangiitis with renal involvement and B-cell lymphoma of the head of the pancreas. MANAGEMENT: The patient received oral prednisolone 15 mg daily for polymyalgia rheumatica along with alendronate 70 mg weekly. The patient received intravenous cyclophosphamide 500 mg and methylprednisolone 500 mg every 2 weeks for her microscopic polyangiitis with renal involvement. For B-cell lymphoma of the head of the pancreas, the patient received cyclophosphamide, doxorubicin, vincristine and prednisolone once monthly.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
8/12. A case of cryptococcal meningoencephalitis and focal cerebral vasculitis with transient immunodeficiency.The AA. report a case of CNS cryptococcosis with vasculopathic complications in a woman in perfect physical conditions. The patient was admitted to hospital after complaining for 20 days of fever, headache and rachialgia. CSF examination revealed hypoglicorrachia (18 mg %), cells 440 mmc (polymorphonuclear leukocytes 90%), brain CT scan negative. During antimycobacterial treatment right hemiparesis appeared; brain CT: hypodense lesion in the posterior brachium of the left inner capsule; at its base the lesion showed a hypodense streak coherent with thromboembolic damage or deep vasculitis. OKT4 lymphocytes were 6% (absolute number: 70). The antimycotic treatment, following the positive reaction of the CSF culture to cryptococcus neoformans, entirely cured the hemiparesis and normalized the CSF while OKT4 lymphocytes rapidly grew.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
9/12. Pericardial disease in rheumatoid arthritis.Six patients with rheumatoid constrictive pericarditis, five seen in a two and one half year period, are described. All patients were male, all had rheumatoid factor, and all had active arthritis. diagnosis was suspected from careful physical examination and confirmed in five patients by cardiac catheterization. pericardiectomy was successful in all five patients on whom it was performed. Rheumatoid constrictive pericarditis should be suspected in any patient with rheumatoid arthritis and unexplained signs of right heart failure.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
10/12. Left atrial myxoma presenting as a systemic vasculitis.Left atrial myxoma has often stimulated systemic vasculitis or a connective tissue disease, but the diagnosis is usually suggested by cardiac symptoms or signs. The present case report is both unusual and instructive in that the patient presented with a painless left foot drop followed by digital vasculitis. Laboratory findings included depressed total hemolytic complement and elevated anti-double-stranded dna. Despite frequent and careful observation, a heart murmur was not appreciated. Because of the unusual features and the patient's frequent military travels, the diagnosis of left atrial myxoma was not established for 8 months. This case history stresses the importance of considering the possibility of atrial myxoma in patients with heart murmurs whose symptoms, physical findings, and laboratory studies could be compatible with a systemic vasculitis.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
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