Diffuse systemic sclerosis with pulmonary involvement and scleroderma renal crisis: report of a rare clinical association
Keywords:
scleroderma renal crisis, pulmonary hypertension,, hiperreninemia, microangiopathyAbstract
INTRODUCTION: Systemic sclerosis (SSc), an immunologic disorder of
unknown aetiology, is characterized by progressive tissue fibrosis, localized or diffuse.
The diffuse sub-type and prolonged course of the disease are associated
with increased risk of visceral involvement, mainly of the lungs and kidneys.
Pulmonary hypertension (PHT) and scleroderma renal crisis (SRC) are not
mutually exclusive, but they are rarely associated; however, both are important
causes of death in these patients. Mortality in SS is four times that of the
general population; mainly in the first 3-5 years, the overall survival rate at 15 years is around 50%.
We report a case of this rare association and present a brief review of
pathophysiology and therapeutics.
CASE REPORT: A 56 year-old female, caucasian, SSc diagnosed 3,5 years
before, treated with vasodilators, oral anticoagulation (due to recurrent deep
venous thrombosis) and corticosteroids (she had fibrosing alveolitis, and
was previously treated with 13 pulses of cyclofosfamide with a favourable response).
She was admitted with fever, arterial hypertension, periorbital oedema and
worsening renal function. A bilateral pleural effusion was diagnosed, without
alveolitis, it was a transudate. ANA e anti-Scl-70 were positive, ANCA and
antiphospholipid being negative. The echocardiogram disclosed PHT and a
pericardial effusion without haemodynamic compromise. Ultrasound of the
kidneys was normal. Oedema and blood pressure improved on captopril;
however renal function rapidly worsened with oliguria, so a renal biopsy (RB)
was made and she began hemodialysis due to uremia and fluid overload.
RB histology was typical of SRC. Renal function had no improvement under
dialysis. In the 29th day of hospitalization she had typical signs of pulmonary
embolism, and treatment began with IV heparin; latter a cardiorespiratory
arrest with assystole ensued, that didn’t responded to cardiopulmonary ressuscitation.
DISCUSSION: SRC is more frequently associated with diffuse SSc and
anti-Scl-70. Its hallmark is an abrupt onset of severe arterial hypertension
and rapidly progressive renal failure with hiperreninemia.
It may coexist a microangiopathic hemolytic anemia similar to hemolyticuremic syndrome.
Therapy with angiotensin converting enzyme inhibitors improved survival
and prognosis, reducing the necessity of dialysis, and sometimes allowing
suspension of renal replacement therapy.
Overlap syndrome / renal vasculitis was considered a possibility due to
poliserositis and recurrent deep venous thrombosis. Corticosteroid theraphy
is likely associated with onset of SRC.
There have been reports linking SRC with the onset of PHT in SSc. Some
authors consider there is a common pathophysiology, namely the coexistence of renal and pulmonary thrombotic microangiopathy with myocardial dysfunction.
Downloads
References
Villas AP et al. Esclerose sistémica – perspectivas actuais. Medicina Interna 2002; 9(2): 111-120.
Steen VD. Treatment of systemic sclerosis. Am J Clin Dermatol. 2001; 2(5): 315-325.
Pla VF et al. Esclerosis sistémica in Ramos-Casals M., Enfermedades autoinmunes sistémicas y reumatológicas, 1ª ed Masson Barcelona 2005: 117-124.
Bonnet F et al. La crise rénale sclérodermique, sept observations et revue de la littérature. Ann Med Interne 1998; 149(5): 243-250.
Steen VD et al. Scleroderma renal crisis. Rheum Dis Clin N Am 2003; 29: 315-333.
Cheung WY et al. Late recurrence of scleroderma renal crisis in a renal transplant recipient despite angiotensin II blockade. Am J Kidney Dis 2005; 45(5): 930-934.
Jego C et al. Apparition d’une crise rénale sclerodermique à l’arrêt d’un traitment par losartan. Rev Med Interne 2003; 24: 272-273.
Kohno K et al. A case of normotensive scleroderma renal crisis after high-dose methylprednisolone treatment. Clinical Nephrology 2000; 53(6): 479-482.
Szigeti N et al. Fatal scleroderma renal crisis caused by gastrointestinal bleeding in a patient with scleroderma, Sjögren’s syndrome and primary billiar cirrhosis overlap. JEADV 2002; 16; 276-279.
Mpofu S et al. An unusual cause of acute renal failure in systemic sclerosis. Ann Rheum Dis 2003; 62: 1133-1134.
Bar J et al. Pulmonary-renal syndrome in systemic sclerosis. Seminars in Arthritis and Rheumatism 2001; 30(6): 403-410.
Gündüz OH et al. Systemic sclerosis with renal crisis and pulmonary hypertension, a report of eleven cases. Arthritis Rheum 2001; 44(7): 1663-1666.
Greenberg SA et al. Inflammatory myopathy associated with mixed connective tissue disease and scleroderma renal crisis. Muscle Nerve 2001; 24: 1562-1566.
Villaverde M et al. Normotensive scleroderma renal crisis. Medicina (B. Aires) 2003; 63(1): 49-50.
Nanke Y et al. Progressive appearance of overlap syndrome together with autoantibodies in a patient with fatal thrombotic microangiopathy. Am J Med Sci 2000; 320(5): 348-351.
Omote A et al. Myeloperoxidase-specific anti-neutrophil cytoplasmic autoantibodies-related scleroderma renal crisis treated with double
filtration plasmapheresis. Internal Medicine 1997; 36:508-513.
Martinez Ara J et al. Progressive systemic sclerosis associated with antimyeloperoxidase ANCA vasculitis with renal and cutaneous involvement. Nefrologia 2000; 20(4): 383-386.
Katrib A et al. Systemic sclerosis and antineutrophil cytoplasmic autoantibody associated renal failure. Rheumatol Int. 1999; 19(1-2): 61-63.
Anders HJ et al. MPO-ANCA-Positive crescentic glomerulonephritis: a distinct entity of scleroderma renal disease? Am J Kidney Dis. 1999; 33(4):e3.
Steen VD et al. Long-term outcomes of scleroderma renal crisis. Ann Intern med 2000; 133(8): 600-603.
Dziadzio M et al. Losartan theraphy for Raynaud’s phenomenon and scleroderma. Arthritis Rheum 1999; 42: 2646-2655.
Kawaguchi Y et al. Angiotensin II in the lesional skin of systemic sclerosis contributes to tissue fibrosis via angiotensina II type 1 receptors. Arthritis Rheum 2004; 50: 216-226.
Caskey FJ et al. Failure of losartan to control blood pressure in scleroderma renal crisis. Lancet 1997; 349: 60.
Gibney EM et al. Kidney transplantation for systemic sclerosis improves survival and may modulate disease activity. Am J Transplant 2004; 4: 2027-2031.
Pham PTT et al. Predictors and risk factors for recurrent scleroderma renal crisis in the kidney allograft: case report and review of the literature. Am J Transplant 2005; 5: 2565-2569.
Denton CP et al. Systemic sclerosis, scleroderma in Rose NR, The Autoimmune Diseases, 4ª ed Elsevier Baltimore 2006: 369-379
Additional Files
Published
How to Cite
Issue
Section
License
This work is licensed under a Creative Commons Attribution 4.0 International License.
Copyright (c) 2023 Medicina Interna
Open Access
