This action might not be possible to undo. Are you sure you want to continue?
IMMUNE MEDIATED COMPLEX SYTEMIC LUPUS ERTHYMATOSUS (SLE) SLE is an automimmune disease in which organs and cells undergo damage mediated by tissue binding autoantibodies and immune complexex. This disease is called the great imitator of almost all diseases. Multiorgan manifestions can be seen in this case that is also consistent in the case. However, diagnostic criteria seen below is not satisfied making this diagnosis unlikely. In this case, the only manifestations that can be seen in the patient that is consistent with the disease are renal affectations, presence of rheumatoid factor, peripheral nervous system involvement seen as sensory deficits and NCV findings of sensorimotor neuropathy mainly of axonal type; mononeuropathy mutliplex and and other non-specific symptoms. Other than this, no other manifestations are seen that can satisfy the criteria for diagnosis.
Rule In RENAL INVOLVEMENT BUN Creatinine ratio: 13:1 • Crea 2.62 BUN 35 Albumin +1 RBC numerous WBC 1-4 Hypertension (-) PULMONARY INVOLVMENT
Rule Out Did not satisfy greater than or equal to 4 criteria ANA: 1:80 Anti DS DNA negative Anti-RNP negative
PAN involves multiorgan system as seen below except for pulmonary involvement which is consistent also in the patient. symptomatology seen in this patient involves organ system specifically Renal and Peripheral Nervous System. Having known that PAN involves multisystem vessels various manifestations can be expected in this disease which can be seen in the case. is a multisystem necrotizing vasculits of small and medium muscualar arteries that commonly involves renal and visceral arteries. Renal manifestations present in the case includes the presence of Acute Renal failure manifested by a BUN Creatinine ratio of 13:1 and microscopic hematuria and albuminuria . Their lesions tend to involve bifurcations and branching of arteries. hypertension . In this case. mononeuropathy mutliplex Others: Fever Weight loss Malaise RF 96IU/ml P-ANCA positive POLYARTERTIS NODOSA (PAN) PAN which is considered a very uncommon disease.PERIPHERAL NERVOUS SYTEM Sensory deficits in both toes and foot NCV: sensorimotor neuropathy mainly of axonal type.
PAN was also thought to lead to Microscopic polyangitis in the long run. Based on manifestations both cannot be fully differentiated aside from the presene of pulmonary involment in microscopic Polyangits seen in 12% of patient and the only difference can be seen upon diagnostic examination particularly immunochemistry and through biopsy seen in histologic evidence of venules involment of vasculitis which cannot be seen in PAN . Other laboratory findings present in the case suggesting PAN is presence of P-ANCA this though is rarely seen. diagnosis of such disease can only be definitive Rule In Rule Out RENAL INVOLVEMENT BUN Creatinine ratio: 13:1 • Crea 2.62 BUN 35 Albumin +1 RBC numerous WBC 1-4 Hypertension (-) PULMONARY INVOLVMENT PERIPHERAL NERVOUS SYTEM Sensory deficits in both toes and foot NCV: sensorimotor neuropathy mainly of axonal type. weight loss. Peripheral Nervous System involvement includes presence of decrease sensitivity of the lower extremities including the toes and ankles and the presence positive NCV findings. and was considered to be under it before. The presence of hepatitis B antigen seen in the case could also be seen in the patient However. body malaise. mononeuropathy mutliplex Others: Fever Weight loss Malaise RF 96IU/ml P-ANCA positive HBsAg reactive P-ANCA ASSOCIATED VASCULITIS MICROSCOPIC POLYANGIITIS This disease is same as PAN.and probably anemia which can be probably due to renal damage producing decrease erythiopeitin levels. On the other hand. Other non-specific symptoms which is present in the case also seen in PAN are fever.
62 BUN 35 Albumin +1 RBC numerous WBC 1-4 Hypertension PERIPHERAL NERVOUS SYTEM Sensory deficits in both toes and foot NCV: sensorimotor neuropathy mainly of axonal type. CVS. malaise. capillaries. In addition. kidneys. mononeuropathy mutliplex Others: Fever Weight loss Malaise RF 96IU/ml P-ANCA positive 75% HBsAg reactive CHURG-STRAUSS SYNDROME Churg-Strauss syndrome is a condition affecting the small and medium-sized arteries. veins and venules. The histopathologic feature of Churg-Strauss syndrome is granulomatous reactions present in the tissues or even the walls themselves. skin. nerves and the GIT. We considered Churg-strauss syndrome due to the fact that it involves basically all organs of the body such as the lungs. The patient's clinical presentation of low grade fever. Churg-strauss was ruled out however due to the fact that it primarily affects the pulmonary system producing a clinical feature of severe asthmatic attack and pulmonary infiltrates. the presence of perinuclear antineutrophil cytoplasmic antibodies (p-ANCA) in 48% of cases is also suggestive of this diseases coupled with the tingling to loss of sensation on both feet (mononeuritis multiplex) which indicate the presence of nerve involvement which is evident in 72% of patients with this disease. To completely rule out this disease entity aside from the clinical presentation biopsy must also be performed. Churg-Strauss Syndrome Rule In • Tingling sensation/Numbness 48% of cases • Fever Rule Out in • Absence of pulmonary affectation such as asthmatic attack and/or pulmonary infiltrates (major presentation of the disease) .Rule In Rule Out RENAL INVOLVEMENT (-) pulmonary involvement BUN Creatinine ratio: 13:1 • Crea 2. and weight loss which is a characteristic of a multisystem disease makes this diagnosis very likely.
the patient has no oral ulcers. However. Biopsy of symptomatic organs in the patient like renal biopsy and muscle/peripheral nerve on the left foot provides the highest diagnostic yield. but positivity is not conclusive and negative ANCAs are not sufficient to reject the diagnosis. What is your most likely impression based on the given information? PRIMARY DIAGNOSIS: POLYARTERITIS NODOSA cannot rule out Microscopic Polyangiitis SECONDARY DIAGNOSIS: Carpal Tunnel Syndrome. most frequently kidneys. Rule in • + hematuria • + red cell cast Rule out • No oral ucer • Bloody nasal discharge • Normal chest X-ray • (-) c-ANCA 4. normal chest X-ray. The patient has hematuria and presence of red cell cast. BIOPSY The diagnosis of PAN is based on the demonstration of characteristic findings of vasculitis on biopsy material of involved organs. . heart and liver. WEGENER GRANULOMATOSIS Wegeners granulomatosis is an inflammation involving respiratory tract and necrotizing vasculitis of small to medium sized vessels. the following must be requested: (1) Biopsy and/or (2) Renal Angiography 1. Hypertension 5. the patient is negative for c-ANCA. It is usually suspected only when a patient has had unexplained symptoms for a long period of time. Determination of ANCAs can aid in the diagnosis. which is 95% positive in patient with Wegeners granulomatosis. In classic PAN there is segmental transmural necrotizing inflammation of arteries of medium to small size. bloody nasal discharge. Any additional work-ups or procedure to request? To diagnose classic polyarteritis nodosa in the patient.• • • Malaise Weight loss p-ANCA positive • Biopsy may be performed in order rule this out optimally. Also.
Fig. . twice weekly for 2 weeks. The study uses iodinated contrast material so allergy to such material must be checked. Plasma exchanges are used as adjunctive therapy with antivirals. Antiviral agents are begun after steroid withdrawal to enhance immunologic clearance of HBV-infected hepatocytes and favor seroconversion. Plasma exchange is performed 3 times per week for 3 weeks. hepatic. Plasma exchanges are stopped once seroconversion from hepatitis B e antigen to hepatitis B e antibody occurs or after clinical recovery is maintained for 2-3 months. and visceral vasculature. In hepatitis B–related PAN. It is recommended that lamivudine be continued for 6 months or stopped at the time of seroconversion to hepatitis B surface antibody. Treatment for Hepatitis B Infection related Polyarteritis Nodosa Pharmacologic Corticosteroids are the cornerstone of treatment. Prednisone (1 mg/kg/d) is administered for the first week. treatment consists of schemes that include corticosteroids with antiviral agents and plasmapheresis. RENAL ANGIOGRAPHY If the patient refuses in the more invasive tissue biopsy or in the absence of easily accessible tissue for biopsy. Cyclophosphamide is not routinely recommended in hepatitis B–related PAN as the use of steroids with cyclophosphamide in these patients has been demonstrated to enhance viral replication. is sufficient to make the diagnosis. the arteriographic demonstration of involved vessels. 6. 1 There is segmental fibrinoid necrosis and thrombotic occlusion of the lumen of this small artery. particularly in the form of aneurysms of small and medium-sized arteries in the renal. Antiviral drugs used include vidarabine or interferon alpha-2b. 2. Steroids are then tapered rapidly and withdrawn at the end of week two. Devise a therapeutic plan for this patient based on the data given to you. and then once weekly.
regular monitoring of blood pressure should be done and target goal of BP level should be <130/80mmHg.Non-pharmacologic Long-Term Monitoring Patients with polyarteritis nodosa (PAN) should be monitored closely for evidence of relapse or symptoms indicating new organ involvement such as heart. vit B. Nonsteroidal anti-inflammatory drugs (NSAIDs) may be of benefit. and omega-3 fatty acids). If the conservative management failed. consume diet rich in fruits and vegetables. maintain of ideal body weight. Non pharmacologic management lifestyle modifications is recommended to prevent progression include stress management and sodium restriction(<2.3 g of sodium/day). . Vitamin B-6 or B-12 supplements are of no proven benefit. reduce dietary saturated fat and cholesterol intake. GI and skin. Hypertension and Acute Renal Failure ACE inhibitor (Captopril 25-200mg BID) is effective in managing hypertension with acute renal failure. The patient’s complete blood count (CBC) should be checked 10-14 days after IV cyclophosphamide is administered and every 2 weeks once oral cyclophosphamide is initiated. magnesium. Carpal tunnel syndrome Conservative management is initially recommended such as splinting the wrist at nighttime for a minimum of 3 weeks. potassium level should be regularly monitored since this drug may increase potassium secretion. most days). steroid injection into the carpal tunnel is recommended. Exercise (aerobic) regularly (at least 30 min/day. However.adequate nutritional intake of minerals and vitamins(vit C. especially for long term benefits.