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Scleroderma with Renal Involvement

Renal Disease due to Scleroderma


Presentation

  • In some cases, SRC was the initial presentation of scleroderma; there were no skin sclerosis during the time that SRC develops [ 8 ].[omicsonline.org]
  • A patient with scleroderma renal disease and pulmonary hypertension who had a successful pregnancy with the use of angiotensin converting enzyme inhibitors is presented.[ncbi.nlm.nih.gov]
  • There is no evidence at present to support the use of angiotensin-converting enzyme inhibitors prophylactically. The mechanisms and significance of chronic renal impairment in scleroderma need to be better defined.[ncbi.nlm.nih.gov]
  • Three years before presentation, she had complained of sausage-like fingers, Raynaud's phenomenon, digital-tip ulcers and decreased wrinkles around her mouth. One year before presentation, she had dyspnea on effort.[jstage.jst.go.jp]
  • Clinical Pearls • How does systemic sclerosis typically present ? Systemic sclerosis or scleroderma has a female preponderance and typically presents in patients who are between 30 and 60 years of age.[blogs.nejm.org]
Raynaud Phenomenon
  • Table 5 presents the characteristics of primary and secondary Raynaud phenomenon.[aafp.org]
  • Patients may have Raynaud’s phenomenon for years before fibrosis starts, 3,7 so clinicians should monitor patients with Raynaud’s closely.[pharmacytimes.com]
  • Raynaud’s Phenomenon Raynaud’s phenomenon and cold intolerance are seen in almost every patient with scleroderma.[hopkinsscleroderma.org]
  • […] doi: 10.1097/BOR.0b013e3283108df7 Raynaud phenomenon, scleroderma, overlap syndromes and other fibrosing syndromes: Edited by John Varga Abstract Author Information Authors Article Metrics Metrics Renal complications are important in scleroderma (systemic[journals.lww.com]
  • Three years before presentation, she had complained of sausage-like fingers, Raynaud's phenomenon, digital-tip ulcers and decreased wrinkles around her mouth. One year before presentation, she had dyspnea on effort.[jstage.jst.go.jp]
Renal Artery Stenosis
  • Renal ultrasound coupled with Doppler excluded the presence of renal artery stenosis and showed small sizes of kidneys.[omicsonline.org]
  • Renal artery stenosis (RAS) is a common complication of APS, leading to renovascular hypertension [ 117 ].[bmcmedicine.biomedcentral.com]
Malnutrition
  • These include fibrosis of the muscle secondary to the scleroderma process, weakness from disuse or wasting from deconditioning and malnutrition.[hopkinsscleroderma.org]
Pleuritic Pain
  • Pleuritis, convincing history of pleuritic pain or rubbing heard by a physician or evidence of pleural effusion, or 2. Pericarditis, documented by electrocardiogram or rub or evidence of pericardial effusion 7. Renal disorder 1.[bmcmedicine.biomedcentral.com]
Vomiting
  • Alkohol dan obat-obatan yang dikenal mengiritasi mukosa lambung dihindari Usually due to either infection or stress this presents as stomach pain vomiting nausea Aside from abdominal pain on the left side Weil die Kopfschmerzen nicht weggegangen sind[banksuenden.eu]
  • He complained about headache scored 4/10 according to digital scale, vomits and epigastric pain. He had neither seizures nor neurological deficits.[omicsonline.org]
  • Case Report A 60-year-old male presented through the outpatient department (OPD) with complaints of shortness of breath on exertion and vomiting for the preceding two months.[jpma.org.pk]
  • On day five, the patient's blood pressure dropped to 130/90 mmHg and symptomatic relief was maintained in terms of headache, nausea and vomiting on day 7.[reumatologiaclinica.org]
  • Once the kidney is severely damaged, the symptoms of CKD can include: swelling around your eyes, called periorbital edema swelling of your legs, called pedal edema fatigue shortness of breath nausea vomiting, especially in the morning and after eating[healthline.com]
Epigastric Pain
  • He complained about headache scored 4/10 according to digital scale, vomits and epigastric pain. He had neither seizures nor neurological deficits.[omicsonline.org]
Hypertension
  • Case Presentation A 33-year-old man, Malagasy was hospitalized in rheumatology department for a management of a malignant hypertension.[omicsonline.org]
  • A patient with scleroderma renal disease and pulmonary hypertension who had a successful pregnancy with the use of angiotensin converting enzyme inhibitors is presented.[ncbi.nlm.nih.gov]
  • (pulmonary arterial hypertension). 10 Patients with limited cutaneous systemic sclerosis have the greatest risk of pulmonary arterial hypertension. 10 Risk factors for severe pulmonary arterial hypertension include limited cutaneous subset, older age,[aafp.org]
  • – a sudden onset of malignant hypertension and renal failure that occurs in a minority of scleroderma patients.[ucsfmed.wordpress.com]
  • Hypertensive crisis. In: Kaplan NM. Kaplan’s Clinical Hypertension. 9th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2006:311. Vaughan CJ, Delanty N. Hypertensive emergencies. Lancet. 2000;356(9227):411-417.[consultant360.com]
Tachycardia
  • Physical examination concluded signs of retinopathy hypertensive staged III, regular sinusal tachycardia, double sound B2 on hearth auscultation, bilateral pleuretic syndrome and oedema on lower limbs.[omicsonline.org]
Hypertensive Retinopathy
  • Acute kidney injury Essentially normal urinalysis with only mild proteinuria and few cells or casts Other associated features: microangiopathic hemolytic anemia, CHF, pulmonary edema, hypertensive retinopathy and encephalopathy and seizure Treatment of[ucsfmed.wordpress.com]
  • In our study 2 patients had malignant hypertension one a BP of 230 / 140mmHg and hypertensive retinopathy (case NO 1) and the other with a BP of 250 / 130mmHg and low consciousness level (case NO 2).[panafrican-med-journal.com]
  • Patients with SRC may exhibit headaches, hypertensive retinopathy associated with visual disturbances and encephalopathy, seizures, fever and general malaise.[academic.oup.com]
  • The consultant Opthalmologist also diagnosed hypertensive retinopathy. Abdominal ultrasonography showed increased bilateral renal parenchymal echogenity, which was consistent with type one renal parenchymal disease.[reumatologiaclinica.org]
  • Patients may report symptoms consistent with hypertensive retinopathy and/or encephalopathy, hematuria, abrupt oliguria/anuria, seizures, and even pulmonary edema. 2,5 This then rapidly progresses to oliguric renal failure, commonly in the absence of[consultant360.com]
Skin Lesion
  • In the admission at hospital, there was unremarkable skin lesion but followed lately by the development of several skin manifestation such as telangiectasia, sclerodactyly, and finished by generalized cutaneous sclerosis [ 2 ].[omicsonline.org]
  • A process which mainly involves small vessels [ 12 ] and manifests as myxoid intimal changes, thrombi, onion skin lesions, and/or fibrointimal proliferation.[panafrican-med-journal.com]
  • The histologic picture of SRC is that of a thrombotic micro-angiopathic process with small vessel involvement manifesting as myxoid intimal changes, thrombi, onion skin lesions and/or fibro-intimal sclerosis.[sjkdt.org]
  • Vascular changes are accompanied by thrombosis, accumulation of myxoid material and later in the disease course, development of onion-skin lesions and/or fibrointimal sclerosis [ 13 ].[bmcmedicine.biomedcentral.com]
Cutaneous Manifestation
  • According to our acknowledges, SRC without cutaneous manifestation is infrequent and few observations have been reported worldwide. U Zwetter in his observation has reported one atypical case of SRC.[omicsonline.org]
  • manifestations. 1,4-6 The magnitude of skin and organ involvement predicts the clinical course and ultimately the morbidity and mortality, with the most dismal prognosis associated with diffuse disease. 2,4 Cases of lcSSc generally are restricted to[consultant360.com]
  • Other cutaneous manifestations include hair loss on involved skin; telangiectasia on the face, buccal mucosa, chest, and hands; and calcinosis cutis ( Online Figures B1 and B2 ).[aafp.org]
Seizure
  • Acute kidney injury Essentially normal urinalysis with only mild proteinuria and few cells or casts Other associated features: microangiopathic hemolytic anemia, CHF, pulmonary edema, hypertensive retinopathy and encephalopathy and seizure Treatment of[ucsfmed.wordpress.com]
  • He had neither seizures nor neurological deficits.[omicsonline.org]
  • These are listed below: Symptom Reason symptom occurs Fatigue, Dizziness, Shortness of breath Low red blood cell count Bruises, Gum/nose bleeds, Minor cuts bleed a lot Low platelet count Confusion, Sleepiness, Seizures Damage to blood cells in the brain[unckidneycenter.org]
  • Patients with SRC may exhibit headaches, hypertensive retinopathy associated with visual disturbances and encephalopathy, seizures, fever and general malaise.[academic.oup.com]
  • Patients may report symptoms consistent with hypertensive retinopathy and/or encephalopathy, hematuria, abrupt oliguria/anuria, seizures, and even pulmonary edema. 2,5 This then rapidly progresses to oliguric renal failure, commonly in the absence of[consultant360.com]
Asthenia
  • His medical history begun two months before his admission by physical asthenia complicated by a weight losing of 20 kg. He didn’t have transit disorder or dysphagia or fever.[omicsonline.org]
Kidney Failure
  • Unless treated promptly, renal crisis leads to kidney failure, a condition in which the kidneys lose their ability to eliminate waste products from the body.[scleroderma.org]
  • It teaches the physician how to recognise the tell-tale signs of the diseases that cause kidney failure and how to confirm their presence in an efficient manner.[books.google.com]
  • Kidney Failure. The kidneys' main function is to eliminate excess fluid and waste material from your blood.[sclero.org]
  • The abrupt onset of kidney failure and severe hypertension in patients with progressive systemic sclerosis. It is treated by controlling blood pressure with angiotensin converting enzyme inhibitors, sometimes in association with other agents.[medical-dictionary.thefreedictionary.com]
Renal Insufficiency
  • Abuelo Springer Science & Business Media, ٠٦‏/١٢‏/٢٠١٢ - 284 من الصفحات Renal Failure, Diagnosis & Treatment is a practical book that provides a comprehensive description of the evaluation of renal insufficiency.[books.google.com]
  • Insufficiency, Chronic Scleroderma, Systemic Scleroderma, Diffuse Scleroderma, Localized Urologic Diseases Renal Insufficiency Connective Tissue Diseases Skin Diseases[clinicaltrials.gov]
  • None of these patients, who were followed for a mean of 10 years after onset of scleroderma, have developed chronic renal insufficiency that progressed to dialysis.[jrheum.org]
  • The black race, malignant high blood pressure, renal insufficiency and systemic manifestation allowed us to report the diagnosis of a systemic sclerosis with renal manifestation.[omicsonline.org]
  • insufficiency 495 Management of renal osteodystrophy 505 Peritoneal dialysis 516 Jonas Bergstrom M D 521 Dialysis access surgery 533 Dialyzers and water treatment 545 Dialysis ultrafiltration and hemofiltration 555 Use of drugs in uremia and dialysis[books.google.com]
Oliguria
  • Four months later the patient was referred to nephrology department for height blood pressure and oliguria. On admission, BP was 230/140 mm Hg and 24 hours urine collection revealed an oliguria with 150 cc / day.[panafrican-med-journal.com]
  • Unexplained malignant hypertensions on systemic scleroderma with a rapid deterioration of renal function associated or no with an oliguria are emergency in nephrology. Despite the absence of skin involvement, SRC should be searched first of all.[omicsonline.org]
  • Patients may report symptoms consistent with hypertensive retinopathy and/or encephalopathy, hematuria, abrupt oliguria/anuria, seizures, and even pulmonary edema. 2,5 This then rapidly progresses to oliguric renal failure, commonly in the absence of[consultant360.com]
  • Pulmonary oedema is also common, resulting from water and salt retention due to large overload and oliguria. Arrythmia, myocarditis and pericarditis, if present, may indicate poorer prognoses [ 10 ].[academic.oup.com]
  • SRC is characterized by an acute, usually symptomatic increase in blood pressure, a rise in serum creatinine levels, oliguria and thrombotic microangiopathy in about 50% of patients.[nature.com]

Workup

Atelectasis
  • Pulmonary tomodensitometry revealed an interstitial syndrome, pulmonary fibrosis with an atelectasis and minimal pleurisy. Pleurisy was transudative and no malignant cells were found.[omicsonline.org]
Normocytic Anemia
  • In paraclinical signs, the blood count showed normochrom normocytic anemia with 10 g/dl of hemoglobin. Lactate dehydrogenases and haptoglobinemia were in theirs normal ranges.[omicsonline.org]
  • CBC revealed a normochromic normocytic anemia with an Hb of 7g/dl, a thrombocytopenia of 99 000 /mm 3 and 2.5% schisocytes in blood smear.[panafrican-med-journal.com]
Creatinine Increased
  • Creatinine test As kidney function declines, your creatinine increases. This protein is also related to muscle mass. Parathyroid hormone (PTH) test The kidney and the parathyroid glands interact through the regulation of calcium and phosphorus.[healthline.com]
Normocytic Normochromic Anemia

Treatment

  • Medsger T, Rodriguez-Reyna T (2006) Scleroderma renal crisis: a high index of suspicion speeds diagnosis and life-saving treatment. South Med J 99: 799-800.[omicsonline.org]
  • For both, completely effective treatment is not available, mainly as a result of the lack of knowledge of its pathogenesis.[academy.theisn.org]
  • Because SRC most commonly stems from the progression of an existing diffuse disease state, the treatment of both is required, although these treatments are not inherently identical. Treatment Angiotensin-converting enzyme inhibitors (ACEIs).[consultant360.com]
  • What is the treatment? Treatments for scleroderma vary according to the problems you have. Various drugs (as well as warm gloves) may help Raynaud’s syndrome, for instance.[edren.org]
  • The advantage of photopheresis treatment is the low frequency of side effects related to treatment. The disadvantages are the practical efforts required and the high treatment cost [ 20, 21 ].[clinmedjournals.org]

Prognosis

  • However, it can help to define prognosis and guide treatment in atypical forms. The prognosis of SRC has dramatically improved with the introduction of angiotensin-converting enzyme inhibitors (ACEi).[ncbi.nlm.nih.gov]
  • Nevertheless, the prognosis is still not good. Survival at one and five years is only 76% and 65%, respectively, for SRC patients treated with ACE inhibitors.[the-rheumatologist.org]
  • Prognosis is worse for males, but may not be related to corticosteroid use, presence of MAHA or severity of renal pathology.[academic.oup.com]
  • No medications appear to be truly effective in patients with aggressive disease and long-term prognosis remains disappointing [ 9 ].[clinmedjournals.org]
  • Blood tests to look for certain antibodies (for instance anti-Scl-70 antibodies, anti-centromere antibodies) may help, and can help predict prognosis.[edren.org]

Etiology

  • CONCLUSION: Patients with diffuse scleroderma without renal crisis rarely have significant increases in serum creatinine or proteinuria that cannot be explained by other etiologies.[jrheum.org]
  • Definition / general Connective tissue disorder of unknown etiology with multiple organ system involvement, symptoms due to excessive collagen deposition and vascular disease Also called scleroderma Clinical features 75% women, usually ages 30 , rare[pathologyoutlines.com]
  • Prophylactic use of ACE inhibitors does not seem to decrease the risk of SRC (observed in other studies) so it may be the changing etiology of SSc over time. Disclosure: M. Turk, None; J. E. Pope, None.[acrabstracts.org]
  • This is an autoimmune disease which etiology was not yet well demonstrated. However, the reported risk factors are mainly diffuse cutaneous manifestations, the black race, the use of high-dose corticosteroids, cold [ 11, 12 ].[omicsonline.org]
  • It has been suggested that the RAAS may be less activated, leading to modest blood pressure (BP) elevation, 2 and that vascular insult and vasoconstriction are the culprits, rather than a renin-mediation etiology.[consultant360.com]

Epidemiology

  • Pathogenesis – thought to involve small interlobular arteries in the kidneys which develop intimal proliferation and spasm à vasculopathy that reduces renal blood flow à activation or RAAS à hypertension and worsened renal dysfunction Epidemiology and[ucsfmed.wordpress.com]
  • The serum creatinine was 831μmol/l equivalent of an estimated glomerular filtration rate 2 ml/min/1.73 m 2 according to chronic kidney disease epidemiology; the urea was 38 mg/dl. Proteinuria was 0.8 g/24 hours.[omicsonline.org]
  • Epidemiology of the Rheumatic Diseases. Oxford, U.K.: Oxford University Press; 1993:192. ... 2. Lawrence RC, Helmick CG, Arnett FC, et al. Estimates of the prevalence of arthritis and selected musculoskeletal disorders in the United States.[aafp.org]
  • Epidemiology of systemic sclerosis: incidence, prevalence, survival, risk factors, malignancy, and environmental triggers. Curr. Opin. Rheumatol. 24, 165–170 (2012). 4. Steen, V. D., Syzd, A., Johnson, J. P., Greenberg, A. & Medsger, T. A. Jr.[nature.com]
  • Epidemiology of systemic sclerosis: incidence, prevalence, survival, risk factors, malignancy, and environmental triggers . Curr. Opin. Rheumatol. 24 , 165–170 (2012). 4. Steen, V. D. , Syzd, A. , Johnson, J. P. , Greenberg, A. & Medsger, T. A. Jr.[nature.com]
Sex distribution
Age distribution

Pathophysiology

  • Pathology The pathophysiology is very similar to malignant nephrosclerosis and is characterized by pronounced constriction of arcuate and interlobular arterioles resulting in focal cortical ischemia.[radiopaedia.org]
  • From basic science and pathophysiology to clinical best practices, Brenner & Rector’s The Kidney is your go-to resource for any stage of your career.[books.google.com]
  • According 2013 American Society for Apheresis guidelines pathophysiology of SSc lends little support to the use of Therapeutic Plasma Exchange [ 10 ].[clinmedjournals.org]
  • "Systematic review: pathophysiology and management of gastrointestinal dysmotility in systemic sclerosis (scleroderma)". Aliment. Pharmacol. Ther. 23 (6): 691–712. doi : 10.1111/j.1365-2036.2006.02804.x.[en.wikipedia.org]
  • Pathology Although the pathophysiology underlying SRC is not completely understood, it is accepted as being related to intimal thickening of the renal interlobular and arcuate arteries as a result of endothelial cell injury and abnormal oxidative stress[consultant360.com]

Prevention

  • Doctors recommend that you get the following vaccinations: pneumococcal vaccine hepatitis B vaccine influenza vaccine H1N1 (swine flu) vaccine You can’t always prevent CKD.[healthline.com]
  • Prevention No preventive measures have been validated in the literature to support predictable, reliable improvement in outcomes.[consultant360.com]
  • Interventions designed for Raynaud’s—protecting fingers and toes from trauma and cold temperatures, and staying warm—may prevent damage.[pharmacytimes.com]
  • To prevent death and serious organ damage TTP requires immediate treatment with plasma exchange (PLEX) – see Figure 5 for a diagram.[unckidneycenter.org]
  • There is limited evidence for treatments to prevent this damage or stop it progressing. Blocking a substance in the blood called endothelin has helped treat some aspects of scleroderma.[clinicaltrials.gov]

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